Pigmentation Disorders

To the Editor:

The use of sunless tanners has become an alternative for individuals who wish to have tan skin without exposure to UV radiation.¹ We present a case of a patient who experienced persistent hyperpigmented patches on the hands months after the use of a sunless tanner containing dihydroxyacetone (DHA), a carbohydrate that reacts with amino acids in the stratum corneum to produce pigments called melanoidins. The hyperpigmentation caused by DHA is due to the Maillard reaction, which is the nonenzymatic glycation of amino groups of proteins by the carbonyl groups of sugar.² Many sunless tanners contain DHA at varying concentrations. Dermatologists should be aware of the benefits and potential side effects of these alternative products so that they can appropriately counsel patients.

A 20-year-old woman with no history of skin disease presented for evaluation of hyperpigmented patches on the dorsal hands of several months’ duration. Physical examination revealed ill-defined hyperpigmented patches on the dorsal fingers without associated scale or erythema (Figure 1). She had a remote history of Hodgkin lymphoma treated with chemotherapy and was in remission for 5 years prior to the current presentation. Her hematologists referred her to dermatology for evaluation, as they did not believe the patches could be related to her chemotherapy given that she had completed the treatment years before.

Products with DHA only confer a sun protection factor of approximately 3⁴; although patients may appear tan, they have no actual decreased risk for sunburn after use. Reports have shown that the use of sunless tanners containing DHA can alter the appearance of melanocytic lesions clinically and has caused pseudochromhidrosis on the palms.^3,5,6 A study performed on a human keratinocyte cell line, HaCaT, showed that DHA can induce DNA damage, cell-cycle block, and apoptosis.⁷ In addition, as described in our case, patients may experience prolonged hyperpigmentation after use.

This case demonstrates the potential for persistent hyperpigmentation months after the use of sunless tanners containing DHA. Asking patients specific questions regarding their history of tanning product use is essential in identifying the pathology. Although a skin biopsy may not be strictly indicated, it may aid diagnosis, especially when the history is unclear. As more dermatologists support the use of sunless tanner, we must be aware of this possible outcome, especially on more cosmetically sensitive areas such as the fingers in this patient. Clinicians should be aware that the US Food and Drug Administration recommends avoiding contact with mucous membranes when applying products containing DHA and also recommends use of a test spot prior to treating the entire body with the product.⁸ Patients must not only be educated on the benefits of using sunless tanners but on the potential side effects with use of these products as well.

To the Editor:

The use of sunless tanners has become an alternative for individuals who wish to have tan skin without exposure to UV radiation.¹ We present a case of a patient who experienced persistent hyperpigmented patches on the hands months after the use of a sunless tanner containing dihydroxyacetone (DHA), a carbohydrate that reacts with amino acids in the stratum corneum to produce pigments called melanoidins. The hyperpigmentation caused by DHA is due to the Maillard reaction, which is the nonenzymatic glycation of amino groups of proteins by the carbonyl groups of sugar.² Many sunless tanners contain DHA at varying concentrations. Dermatologists should be aware of the benefits and potential side effects of these alternative products so that they can appropriately counsel patients.

A 20-year-old woman with no history of skin disease presented for evaluation of hyperpigmented patches on the dorsal hands of several months’ duration. Physical examination revealed ill-defined hyperpigmented patches on the dorsal fingers without associated scale or erythema (Figure 1). She had a remote history of Hodgkin lymphoma treated with chemotherapy and was in remission for 5 years prior to the current presentation. Her hematologists referred her to dermatology for evaluation, as they did not believe the patches could be related to her chemotherapy given that she had completed the treatment years before.

Products with DHA only confer a sun protection factor of approximately 3⁴; although patients may appear tan, they have no actual decreased risk for sunburn after use. Reports have shown that the use of sunless tanners containing DHA can alter the appearance of melanocytic lesions clinically and has caused pseudochromhidrosis on the palms.^3,5,6 A study performed on a human keratinocyte cell line, HaCaT, showed that DHA can induce DNA damage, cell-cycle block, and apoptosis.⁷ In addition, as described in our case, patients may experience prolonged hyperpigmentation after use.

This case demonstrates the potential for persistent hyperpigmentation months after the use of sunless tanners containing DHA. Asking patients specific questions regarding their history of tanning product use is essential in identifying the pathology. Although a skin biopsy may not be strictly indicated, it may aid diagnosis, especially when the history is unclear. As more dermatologists support the use of sunless tanner, we must be aware of this possible outcome, especially on more cosmetically sensitive areas such as the fingers in this patient. Clinicians should be aware that the US Food and Drug Administration recommends avoiding contact with mucous membranes when applying products containing DHA and also recommends use of a test spot prior to treating the entire body with the product.⁸ Patients must not only be educated on the benefits of using sunless tanners but on the potential side effects with use of these products as well.

To the Editor:

The use of sunless tanners has become an alternative for individuals who wish to have tan skin without exposure to UV radiation.¹ We present a case of a patient who experienced persistent hyperpigmented patches on the hands months after the use of a sunless tanner containing dihydroxyacetone (DHA), a carbohydrate that reacts with amino acids in the stratum corneum to produce pigments called melanoidins. The hyperpigmentation caused by DHA is due to the Maillard reaction, which is the nonenzymatic glycation of amino groups of proteins by the carbonyl groups of sugar.² Many sunless tanners contain DHA at varying concentrations. Dermatologists should be aware of the benefits and potential side effects of these alternative products so that they can appropriately counsel patients.

A 20-year-old woman with no history of skin disease presented for evaluation of hyperpigmented patches on the dorsal hands of several months’ duration. Physical examination revealed ill-defined hyperpigmented patches on the dorsal fingers without associated scale or erythema (Figure 1). She had a remote history of Hodgkin lymphoma treated with chemotherapy and was in remission for 5 years prior to the current presentation. Her hematologists referred her to dermatology for evaluation, as they did not believe the patches could be related to her chemotherapy given that she had completed the treatment years before.

Products with DHA only confer a sun protection factor of approximately 3⁴; although patients may appear tan, they have no actual decreased risk for sunburn after use. Reports have shown that the use of sunless tanners containing DHA can alter the appearance of melanocytic lesions clinically and has caused pseudochromhidrosis on the palms.^3,5,6 A study performed on a human keratinocyte cell line, HaCaT, showed that DHA can induce DNA damage, cell-cycle block, and apoptosis.⁷ In addition, as described in our case, patients may experience prolonged hyperpigmentation after use.

This case demonstrates the potential for persistent hyperpigmentation months after the use of sunless tanners containing DHA. Asking patients specific questions regarding their history of tanning product use is essential in identifying the pathology. Although a skin biopsy may not be strictly indicated, it may aid diagnosis, especially when the history is unclear. As more dermatologists support the use of sunless tanner, we must be aware of this possible outcome, especially on more cosmetically sensitive areas such as the fingers in this patient. Clinicians should be aware that the US Food and Drug Administration recommends avoiding contact with mucous membranes when applying products containing DHA and also recommends use of a test spot prior to treating the entire body with the product.⁸ Patients must not only be educated on the benefits of using sunless tanners but on the potential side effects with use of these products as well.

The histopathologic diagnosis of vitiligo is classically understood as the absence of melanocytes and melanin in the skin biopsy.¹ It is difficult for a pathologist to establish the absolute absence of melanocytes and melanin in a skin biopsy. Therefore, we need to take into consideration many variables when we face the possibility to biopsy a vitiligo lesion.

The basis of the clinical diagnosis of vitiligo is the appearance of achromic lesions in periorificial and acral areas; however, sometimes it is difficult to differentiate between an achromic or hypochromic lesion. Although Wood light is of great help in these circumstances, it still can be difficult to make the diagnosis with certainty.

In other cases, the lesions do not present a classic distribution of vitiligo, and other differential diagnoses are considered. For example, if we see a single hypochromic or achromic lesion in a young child, then the main differential diagnosis would be achromic nevus. If there are multiple lesions, then we may consider progressive macular hypomelanosis, postinflammatory hypopigmentation, and hypopigmented mycosis fungoides. In genital lesions, the differential diagnosis between initial lichen sclerosus and vitiligo also can be considered. Finally, we must always bear in mind that both sarcoidosis and Hansen disease can appear as achromic or hypochromic lesions.

The histologic diagnosis of vitiligo in a completely constituted lesion implies the total loss of melanocytes and melanin in the epidermis. Additional histologic findings are described at the edge of the advanced border, such as the presence of melanocytes that have increased in size with large dendrites and lymphoid infiltrate. In perilesional skin, vacuolated keratinocytes and Langerhans cells have increased in number and repositioned in the basal layer, with visible degeneration of nerves and sweat glands. Lymphocytes also can be found in contact with the melanocytes.² It is important to note that in addition to these histologic findings, it is common to find spongiosis, mononuclear superficial perivascular inflammatory infiltrate, and melanophages in biopsies of vitiligo.³

Given that ensuring the absence of melanocytes is central to diagnosis and melanocytes can be difficult to identify or differentiate from repositioned Langerhans cells in the basal layer with hematoxylin and eosin stain, immunohistochemical techniques must be performed every time we are dealing with vitiligo biopsies. Although there are no studies comparing the diagnostic value of the different immunohistochemical techniques in vitiligo, dihydroxyphenylalanine (DOPA) seems to be a good option, as it will only mark active melanocytes. Human melanoma black 45 (HMB-45), anti-TYRP1 (Mel-5), and antimelanoma gp 100 antibody (NKI/beteb) also have been used. Some authors recommend the use of pan melanoma because it includes 3 markers—HMB-45, tyrosinase, and Mart-1. Currently, SRY-related HMG-box10 (SOX10) seems to be a good option, as it is a nuclear marker that makes it easier to differentiate melanocytes from pigmented keratinocytes.⁴

Establishing a complete absence of melanocytes in the lesions or finding there are melanocytes but they are inactivated is key to evaluating the pathogenesis of vitiligo and directly affects the histologic diagnosis and eventually even the treatment. Le Poole et al⁵ used a panel of 17 monoclonal antibodies and a polyclonal antibody in lesions of 12 patients with vitiligo without identifying the presence of melanocytes. They concluded that there are no melanocytes in lesions of vitiligo.⁵

In a subsequent study with a larger number of patients, Kim et al² found melanocytes that marked with NKI/beteb and Mart-1 in 12 of 100 patients with vitiligo. They also showed melanocytes by electron microscopy in lesional skin of 1 of 3 patients with vitiligo.² Tobin et al⁶ managed to grow melanocytes from skin with vitiligo and confirmed the presence of melanin in basal keratinocytes of lesions of stable vitiligo. From this evidence we can conclude that the absence of melanocytes and melanin in the epidermis confirms the diagnosis of vitiligo; however, the opposite is not true—that is, the presence of melanocytes or melanin in a skin biopsy does not rule out the diagnosis of vitiligo.

Taking this information into consideration, we can understand that if our differential diagnosis is a dermatosis that requires the evaluation of the number of melanocytes as a fundamental diagnostic clue (eg, postinflammatory hypopigmentation), the biopsy will probably not be useful. On the other hand, when our differential diagnosis has characteristic diagnostic findings independent of the number of melanocytes or the presence of melanin, the biopsy will be useful (eg, hypopigmented mycosis fungoides).

Thus, we can understand why the histologic differentiation between vitiligo, pityriasis alba, postinflammatory hypopigmentation, and progressive macular hypopigmentation is difficult. The histology images of these 4 diseases may show different degrees of melanocyte and melanin decrease, spongiosis, and in the superficial dermis melanophages and mononuclear inflammatory infiltrate.⁷

Nevus depigmentosus also may generate diagnostic confusion with vitiligo. Although it is unilateral and usually congenital, it can appear as late as 3 years of age, leading to an initial clinical differential diagnosis of vitiligo. The histologic findings in this nevus also overlap with vitiligo. The characteristic findings are presence of melanocytes and decreased pigment in the keratinocytes compared with perilesional skin. Therefore, a biopsy is not a solution to this diagnostic dilemma.⁸

In all the differentials named, the solution to the diagnostic doubt is not based on the histologic findings but on the clinical evolution of the patients. In cases of vitiligo, the lesions will become more evident in the evolution. They will eventually disappear in pityriasis alba, postinflammatory hypopigmentation, and progressive macular hypopigmentation and will remain unchanged in nevus depigmentosus. It is important, especially when we are dealing with concerned parents/guardians, to convey the importance of assessing the evolution of the disease as the main diagnostic procedure. Even though a biopsy is minimally invasive, it is usually stressful on children, it may leave sequelae, and above all it will not contribute to the diagnosis in this clinical context.

There are other clinical circumstances in the scenario of hypochromic or achromic lesions in which the biopsy will be useful: If we consider an initial genital lichen sclerosus vs vitiligo. In lichen sclerosus the biopsy will show dermal hyalinosis and interphase changes; absence of both will support vitiligo. If we need to differentiate hypopigmented mycosis fungoides from vitiligo, we will find an infiltrate of pleomorphic lymphocytes in the epidermis and dermis in the former and an absence of these findings in vitiligo. Finally, if we find granulomas in a biopsy of an achromic or hypopigmented lesion, we may be dealing with hypopigmented sarcoidosis or Hansen disease.

It also is important to choose the best site to perform the biopsy to have the best chance at diagnosing vitiligo histologically. As already described, in the edges and in the perilesional skin we can find remnant melanocytes, Langerhans cells, and interphase changes that do not allow us to clearly evaluate the main change that is the loss of melanocytes and melanin. In fact, a biopsy of the edge of a vitiligo macula can lead to confusion. For example, if the differential diagnosis is lichen sclerosus and the image we see in the biopsy of the edge of a vitiligo lesion is an interface reaction, we can interpret it as a finding that favors lichen sclerosus. In this way, it is better to biopsy the center of a well-constituted vitiligo lesion where we have the best chance to assess the absence of melanin and melanocytes.

The vitiligo differential diagnosis can be divided into 2 groups: entities that are difficult to differentiate from vitiligo histologically (ie, pityriasis alba, postinflammatory hypopigmentation, progressive macular hypopigmentation, nevus depigmentosus) and entities that are easily distinguishable from vitiligo histologically (ie, lichen sclerosus, mycosis fungoides, sarcoidosis, leprosy). If our differential diagnosis was found in the first group, the final diagnosis should be based on the evolution of the patient. If it was in the second group, a biopsy of the center of the lesion will be useful and may allow us to reach a definitive diagnosis.

The histopathologic diagnosis of vitiligo is classically understood as the absence of melanocytes and melanin in the skin biopsy.¹ It is difficult for a pathologist to establish the absolute absence of melanocytes and melanin in a skin biopsy. Therefore, we need to take into consideration many variables when we face the possibility to biopsy a vitiligo lesion.

The basis of the clinical diagnosis of vitiligo is the appearance of achromic lesions in periorificial and acral areas; however, sometimes it is difficult to differentiate between an achromic or hypochromic lesion. Although Wood light is of great help in these circumstances, it still can be difficult to make the diagnosis with certainty.

In other cases, the lesions do not present a classic distribution of vitiligo, and other differential diagnoses are considered. For example, if we see a single hypochromic or achromic lesion in a young child, then the main differential diagnosis would be achromic nevus. If there are multiple lesions, then we may consider progressive macular hypomelanosis, postinflammatory hypopigmentation, and hypopigmented mycosis fungoides. In genital lesions, the differential diagnosis between initial lichen sclerosus and vitiligo also can be considered. Finally, we must always bear in mind that both sarcoidosis and Hansen disease can appear as achromic or hypochromic lesions.

The histologic diagnosis of vitiligo in a completely constituted lesion implies the total loss of melanocytes and melanin in the epidermis. Additional histologic findings are described at the edge of the advanced border, such as the presence of melanocytes that have increased in size with large dendrites and lymphoid infiltrate. In perilesional skin, vacuolated keratinocytes and Langerhans cells have increased in number and repositioned in the basal layer, with visible degeneration of nerves and sweat glands. Lymphocytes also can be found in contact with the melanocytes.² It is important to note that in addition to these histologic findings, it is common to find spongiosis, mononuclear superficial perivascular inflammatory infiltrate, and melanophages in biopsies of vitiligo.³

Given that ensuring the absence of melanocytes is central to diagnosis and melanocytes can be difficult to identify or differentiate from repositioned Langerhans cells in the basal layer with hematoxylin and eosin stain, immunohistochemical techniques must be performed every time we are dealing with vitiligo biopsies. Although there are no studies comparing the diagnostic value of the different immunohistochemical techniques in vitiligo, dihydroxyphenylalanine (DOPA) seems to be a good option, as it will only mark active melanocytes. Human melanoma black 45 (HMB-45), anti-TYRP1 (Mel-5), and antimelanoma gp 100 antibody (NKI/beteb) also have been used. Some authors recommend the use of pan melanoma because it includes 3 markers—HMB-45, tyrosinase, and Mart-1. Currently, SRY-related HMG-box10 (SOX10) seems to be a good option, as it is a nuclear marker that makes it easier to differentiate melanocytes from pigmented keratinocytes.⁴

Establishing a complete absence of melanocytes in the lesions or finding there are melanocytes but they are inactivated is key to evaluating the pathogenesis of vitiligo and directly affects the histologic diagnosis and eventually even the treatment. Le Poole et al⁵ used a panel of 17 monoclonal antibodies and a polyclonal antibody in lesions of 12 patients with vitiligo without identifying the presence of melanocytes. They concluded that there are no melanocytes in lesions of vitiligo.⁵

In a subsequent study with a larger number of patients, Kim et al² found melanocytes that marked with NKI/beteb and Mart-1 in 12 of 100 patients with vitiligo. They also showed melanocytes by electron microscopy in lesional skin of 1 of 3 patients with vitiligo.² Tobin et al⁶ managed to grow melanocytes from skin with vitiligo and confirmed the presence of melanin in basal keratinocytes of lesions of stable vitiligo. From this evidence we can conclude that the absence of melanocytes and melanin in the epidermis confirms the diagnosis of vitiligo; however, the opposite is not true—that is, the presence of melanocytes or melanin in a skin biopsy does not rule out the diagnosis of vitiligo.

Taking this information into consideration, we can understand that if our differential diagnosis is a dermatosis that requires the evaluation of the number of melanocytes as a fundamental diagnostic clue (eg, postinflammatory hypopigmentation), the biopsy will probably not be useful. On the other hand, when our differential diagnosis has characteristic diagnostic findings independent of the number of melanocytes or the presence of melanin, the biopsy will be useful (eg, hypopigmented mycosis fungoides).

Thus, we can understand why the histologic differentiation between vitiligo, pityriasis alba, postinflammatory hypopigmentation, and progressive macular hypopigmentation is difficult. The histology images of these 4 diseases may show different degrees of melanocyte and melanin decrease, spongiosis, and in the superficial dermis melanophages and mononuclear inflammatory infiltrate.⁷

Nevus depigmentosus also may generate diagnostic confusion with vitiligo. Although it is unilateral and usually congenital, it can appear as late as 3 years of age, leading to an initial clinical differential diagnosis of vitiligo. The histologic findings in this nevus also overlap with vitiligo. The characteristic findings are presence of melanocytes and decreased pigment in the keratinocytes compared with perilesional skin. Therefore, a biopsy is not a solution to this diagnostic dilemma.⁸

In all the differentials named, the solution to the diagnostic doubt is not based on the histologic findings but on the clinical evolution of the patients. In cases of vitiligo, the lesions will become more evident in the evolution. They will eventually disappear in pityriasis alba, postinflammatory hypopigmentation, and progressive macular hypopigmentation and will remain unchanged in nevus depigmentosus. It is important, especially when we are dealing with concerned parents/guardians, to convey the importance of assessing the evolution of the disease as the main diagnostic procedure. Even though a biopsy is minimally invasive, it is usually stressful on children, it may leave sequelae, and above all it will not contribute to the diagnosis in this clinical context.

There are other clinical circumstances in the scenario of hypochromic or achromic lesions in which the biopsy will be useful: If we consider an initial genital lichen sclerosus vs vitiligo. In lichen sclerosus the biopsy will show dermal hyalinosis and interphase changes; absence of both will support vitiligo. If we need to differentiate hypopigmented mycosis fungoides from vitiligo, we will find an infiltrate of pleomorphic lymphocytes in the epidermis and dermis in the former and an absence of these findings in vitiligo. Finally, if we find granulomas in a biopsy of an achromic or hypopigmented lesion, we may be dealing with hypopigmented sarcoidosis or Hansen disease.

It also is important to choose the best site to perform the biopsy to have the best chance at diagnosing vitiligo histologically. As already described, in the edges and in the perilesional skin we can find remnant melanocytes, Langerhans cells, and interphase changes that do not allow us to clearly evaluate the main change that is the loss of melanocytes and melanin. In fact, a biopsy of the edge of a vitiligo macula can lead to confusion. For example, if the differential diagnosis is lichen sclerosus and the image we see in the biopsy of the edge of a vitiligo lesion is an interface reaction, we can interpret it as a finding that favors lichen sclerosus. In this way, it is better to biopsy the center of a well-constituted vitiligo lesion where we have the best chance to assess the absence of melanin and melanocytes.

The vitiligo differential diagnosis can be divided into 2 groups: entities that are difficult to differentiate from vitiligo histologically (ie, pityriasis alba, postinflammatory hypopigmentation, progressive macular hypopigmentation, nevus depigmentosus) and entities that are easily distinguishable from vitiligo histologically (ie, lichen sclerosus, mycosis fungoides, sarcoidosis, leprosy). If our differential diagnosis was found in the first group, the final diagnosis should be based on the evolution of the patient. If it was in the second group, a biopsy of the center of the lesion will be useful and may allow us to reach a definitive diagnosis.

The histopathologic diagnosis of vitiligo is classically understood as the absence of melanocytes and melanin in the skin biopsy.¹ It is difficult for a pathologist to establish the absolute absence of melanocytes and melanin in a skin biopsy. Therefore, we need to take into consideration many variables when we face the possibility to biopsy a vitiligo lesion.

The basis of the clinical diagnosis of vitiligo is the appearance of achromic lesions in periorificial and acral areas; however, sometimes it is difficult to differentiate between an achromic or hypochromic lesion. Although Wood light is of great help in these circumstances, it still can be difficult to make the diagnosis with certainty.

In other cases, the lesions do not present a classic distribution of vitiligo, and other differential diagnoses are considered. For example, if we see a single hypochromic or achromic lesion in a young child, then the main differential diagnosis would be achromic nevus. If there are multiple lesions, then we may consider progressive macular hypomelanosis, postinflammatory hypopigmentation, and hypopigmented mycosis fungoides. In genital lesions, the differential diagnosis between initial lichen sclerosus and vitiligo also can be considered. Finally, we must always bear in mind that both sarcoidosis and Hansen disease can appear as achromic or hypochromic lesions.

The histologic diagnosis of vitiligo in a completely constituted lesion implies the total loss of melanocytes and melanin in the epidermis. Additional histologic findings are described at the edge of the advanced border, such as the presence of melanocytes that have increased in size with large dendrites and lymphoid infiltrate. In perilesional skin, vacuolated keratinocytes and Langerhans cells have increased in number and repositioned in the basal layer, with visible degeneration of nerves and sweat glands. Lymphocytes also can be found in contact with the melanocytes.² It is important to note that in addition to these histologic findings, it is common to find spongiosis, mononuclear superficial perivascular inflammatory infiltrate, and melanophages in biopsies of vitiligo.³

Given that ensuring the absence of melanocytes is central to diagnosis and melanocytes can be difficult to identify or differentiate from repositioned Langerhans cells in the basal layer with hematoxylin and eosin stain, immunohistochemical techniques must be performed every time we are dealing with vitiligo biopsies. Although there are no studies comparing the diagnostic value of the different immunohistochemical techniques in vitiligo, dihydroxyphenylalanine (DOPA) seems to be a good option, as it will only mark active melanocytes. Human melanoma black 45 (HMB-45), anti-TYRP1 (Mel-5), and antimelanoma gp 100 antibody (NKI/beteb) also have been used. Some authors recommend the use of pan melanoma because it includes 3 markers—HMB-45, tyrosinase, and Mart-1. Currently, SRY-related HMG-box10 (SOX10) seems to be a good option, as it is a nuclear marker that makes it easier to differentiate melanocytes from pigmented keratinocytes.⁴

Establishing a complete absence of melanocytes in the lesions or finding there are melanocytes but they are inactivated is key to evaluating the pathogenesis of vitiligo and directly affects the histologic diagnosis and eventually even the treatment. Le Poole et al⁵ used a panel of 17 monoclonal antibodies and a polyclonal antibody in lesions of 12 patients with vitiligo without identifying the presence of melanocytes. They concluded that there are no melanocytes in lesions of vitiligo.⁵

In a subsequent study with a larger number of patients, Kim et al² found melanocytes that marked with NKI/beteb and Mart-1 in 12 of 100 patients with vitiligo. They also showed melanocytes by electron microscopy in lesional skin of 1 of 3 patients with vitiligo.² Tobin et al⁶ managed to grow melanocytes from skin with vitiligo and confirmed the presence of melanin in basal keratinocytes of lesions of stable vitiligo. From this evidence we can conclude that the absence of melanocytes and melanin in the epidermis confirms the diagnosis of vitiligo; however, the opposite is not true—that is, the presence of melanocytes or melanin in a skin biopsy does not rule out the diagnosis of vitiligo.

Taking this information into consideration, we can understand that if our differential diagnosis is a dermatosis that requires the evaluation of the number of melanocytes as a fundamental diagnostic clue (eg, postinflammatory hypopigmentation), the biopsy will probably not be useful. On the other hand, when our differential diagnosis has characteristic diagnostic findings independent of the number of melanocytes or the presence of melanin, the biopsy will be useful (eg, hypopigmented mycosis fungoides).

Thus, we can understand why the histologic differentiation between vitiligo, pityriasis alba, postinflammatory hypopigmentation, and progressive macular hypopigmentation is difficult. The histology images of these 4 diseases may show different degrees of melanocyte and melanin decrease, spongiosis, and in the superficial dermis melanophages and mononuclear inflammatory infiltrate.⁷

Nevus depigmentosus also may generate diagnostic confusion with vitiligo. Although it is unilateral and usually congenital, it can appear as late as 3 years of age, leading to an initial clinical differential diagnosis of vitiligo. The histologic findings in this nevus also overlap with vitiligo. The characteristic findings are presence of melanocytes and decreased pigment in the keratinocytes compared with perilesional skin. Therefore, a biopsy is not a solution to this diagnostic dilemma.⁸

In all the differentials named, the solution to the diagnostic doubt is not based on the histologic findings but on the clinical evolution of the patients. In cases of vitiligo, the lesions will become more evident in the evolution. They will eventually disappear in pityriasis alba, postinflammatory hypopigmentation, and progressive macular hypopigmentation and will remain unchanged in nevus depigmentosus. It is important, especially when we are dealing with concerned parents/guardians, to convey the importance of assessing the evolution of the disease as the main diagnostic procedure. Even though a biopsy is minimally invasive, it is usually stressful on children, it may leave sequelae, and above all it will not contribute to the diagnosis in this clinical context.

There are other clinical circumstances in the scenario of hypochromic or achromic lesions in which the biopsy will be useful: If we consider an initial genital lichen sclerosus vs vitiligo. In lichen sclerosus the biopsy will show dermal hyalinosis and interphase changes; absence of both will support vitiligo. If we need to differentiate hypopigmented mycosis fungoides from vitiligo, we will find an infiltrate of pleomorphic lymphocytes in the epidermis and dermis in the former and an absence of these findings in vitiligo. Finally, if we find granulomas in a biopsy of an achromic or hypopigmented lesion, we may be dealing with hypopigmented sarcoidosis or Hansen disease.

It also is important to choose the best site to perform the biopsy to have the best chance at diagnosing vitiligo histologically. As already described, in the edges and in the perilesional skin we can find remnant melanocytes, Langerhans cells, and interphase changes that do not allow us to clearly evaluate the main change that is the loss of melanocytes and melanin. In fact, a biopsy of the edge of a vitiligo macula can lead to confusion. For example, if the differential diagnosis is lichen sclerosus and the image we see in the biopsy of the edge of a vitiligo lesion is an interface reaction, we can interpret it as a finding that favors lichen sclerosus. In this way, it is better to biopsy the center of a well-constituted vitiligo lesion where we have the best chance to assess the absence of melanin and melanocytes.

The vitiligo differential diagnosis can be divided into 2 groups: entities that are difficult to differentiate from vitiligo histologically (ie, pityriasis alba, postinflammatory hypopigmentation, progressive macular hypopigmentation, nevus depigmentosus) and entities that are easily distinguishable from vitiligo histologically (ie, lichen sclerosus, mycosis fungoides, sarcoidosis, leprosy). If our differential diagnosis was found in the first group, the final diagnosis should be based on the evolution of the patient. If it was in the second group, a biopsy of the center of the lesion will be useful and may allow us to reach a definitive diagnosis.

LAHAINA, HAWAII – Reassuring evidence of the safety of oral propranolol for treatment of complicated infantile hemangiomas in patients with PHACE syndrome comes from a recent multicenter study.

Bruce Jancin/MDedge News

Oral propranolol is now well-ensconced as first-line therapy for complicated infantile hemangiomas in otherwise healthy children. However, the beta-blocker’s use in PHACE (Posterior fossa malformations, Hemangiomas, Arterial anomalies, Cardiac defects, and Eye abnormalities) syndrome has been controversial, with concerns raised by some that it might raise the risk for arterial ischemic stroke. Not so, Moise L. Levy, MD, said at the Hawaii Dermatology Seminar provided by Global Academy for Medical Education/Skin Disease Education Foundation.

“I’m not suggesting you use propranolol with reckless abandon in this population, but this stroke concern is something that should be put to bed based on this study,” advised Dr. Levy, professor of dermatology and pediatrics at Dell Medical School in Austin, Tex., and physician-in-chief at Dell Children’s Medical Center.

PHACE syndrome is characterized by large, thick, plaque-like hemangiomas greater than 5 cm in size, most commonly on the face, although they can be located elsewhere.

“There was concern that if you found severely altered cerebrovascular arterial flow and you put a kid on a beta-blocker you might be causing some harm. But what I will tell you is that in this recently published paper this was not in fact an issue,” he said.

Dr. Levy was not an investigator in the multicenter retrospective study, which included 76 patients with PHACE syndrome treated for infantile hemangioma with oral propranolol at 0.3 mg/kg per dose or more at 11 academic tertiary care pediatric dermatology clinics. Treatment started at a median age of 56 days.

There were no strokes, TIAs, cardiovascular events, or other significant problems associated with treatment. Twenty-nine children experienced mild adverse events: minor gastrointestinal or respiratory symptoms, and sleep disturbances were threefold more frequent than reported with placebo in another study. The investigators noted that the safety experience in their PHACE syndrome population compared favorably with that in 726 infants without PHACE syndrome who received oral propranolol for hemangiomas, where the incidence of serious adverse events on treatment was 0.4% (JAMA Dermatol. 2019 Dec 11. doi: 10.1001/jamadermatol.2019.3839).

 

‘Hemangiomas – but we were taught that they go away’

Dr. Levy gave a shout-out to the American Academy of Pediatrics for publishing interdisciplinary expert consensus-based practice guidelines for the management of infantile hemangiomas, which he praised as “quite well done” (Pediatrics. 2019 Jan;143[1]. pii: e20183475. doi: 10.1542/peds.2018-3475).

Following release of the guidelines last year, he and other pediatric vascular anomalies experts saw an uptick in referrals from general pediatricians, which has since tapered off.

“It’s probably like for all of us: We read an article, it’s fresh on the mind, then you forget about the article and what you’ve read. So we need a little reinforcement from a learning perspective. This is a great article,” he said.

The guidelines debunk as myth the classic teaching that infantile hemangiomas go away. Explicit information is provided about the high-risk anatomic sites warranting consideration for early referral, including the periocular, lumbosacral, and perineal areas, the lip, and lower face.

“The major point is early identification of those lesions requiring evaluation and intervention. Hemangiomas generally speaking are at their ultimate size by 3-5 months of age. The bottom line is if you think something needs to be done, please send that patient, or act upon that patient, sooner rather than later. I can’t tell you how many cases of hemangiomas I’ve seen when the kid is 18 months of age, 3 years of age, 5 years, with a large area of redundant skin, scarring, or something of that sort, and it would have been really nice to have seen them earlier and acted upon them then,” the pediatric dermatologist said.

The guidelines recommend intervention or referral by 1 month of age, ideally. Guidance is provided about the use of oral propranolol as first-line therapy.

“Propranolol is something that has been a real game changer for us,” he noted. “Many people continue to be worried about side effects in using this, particularly in the young childhood population, but this paper shows pretty clearly that hypotension or bradycardia is not a real concern. I never hospitalize these patients for propranolol therapy except in high-risk populations: very preemie, any history of breathing problems. We check the blood pressure and heart rate at baseline, again at 7-10 days, and at every visit. We’ve never found any significant drop in blood pressure.”

Dr. Levy reported financial relationships with half a dozen pharmaceutical companies, none relevant to his presentation.

SDEF/Global Academy for Medical Education and this news organization are owned by the same parent company.

[email protected]

LAHAINA, HAWAII – Reassuring evidence of the safety of oral propranolol for treatment of complicated infantile hemangiomas in patients with PHACE syndrome comes from a recent multicenter study.

Bruce Jancin/MDedge News

Oral propranolol is now well-ensconced as first-line therapy for complicated infantile hemangiomas in otherwise healthy children. However, the beta-blocker’s use in PHACE (Posterior fossa malformations, Hemangiomas, Arterial anomalies, Cardiac defects, and Eye abnormalities) syndrome has been controversial, with concerns raised by some that it might raise the risk for arterial ischemic stroke. Not so, Moise L. Levy, MD, said at the Hawaii Dermatology Seminar provided by Global Academy for Medical Education/Skin Disease Education Foundation.

“I’m not suggesting you use propranolol with reckless abandon in this population, but this stroke concern is something that should be put to bed based on this study,” advised Dr. Levy, professor of dermatology and pediatrics at Dell Medical School in Austin, Tex., and physician-in-chief at Dell Children’s Medical Center.

PHACE syndrome is characterized by large, thick, plaque-like hemangiomas greater than 5 cm in size, most commonly on the face, although they can be located elsewhere.

“There was concern that if you found severely altered cerebrovascular arterial flow and you put a kid on a beta-blocker you might be causing some harm. But what I will tell you is that in this recently published paper this was not in fact an issue,” he said.

Dr. Levy was not an investigator in the multicenter retrospective study, which included 76 patients with PHACE syndrome treated for infantile hemangioma with oral propranolol at 0.3 mg/kg per dose or more at 11 academic tertiary care pediatric dermatology clinics. Treatment started at a median age of 56 days.

There were no strokes, TIAs, cardiovascular events, or other significant problems associated with treatment. Twenty-nine children experienced mild adverse events: minor gastrointestinal or respiratory symptoms, and sleep disturbances were threefold more frequent than reported with placebo in another study. The investigators noted that the safety experience in their PHACE syndrome population compared favorably with that in 726 infants without PHACE syndrome who received oral propranolol for hemangiomas, where the incidence of serious adverse events on treatment was 0.4% (JAMA Dermatol. 2019 Dec 11. doi: 10.1001/jamadermatol.2019.3839).

 

‘Hemangiomas – but we were taught that they go away’

Dr. Levy gave a shout-out to the American Academy of Pediatrics for publishing interdisciplinary expert consensus-based practice guidelines for the management of infantile hemangiomas, which he praised as “quite well done” (Pediatrics. 2019 Jan;143[1]. pii: e20183475. doi: 10.1542/peds.2018-3475).

Following release of the guidelines last year, he and other pediatric vascular anomalies experts saw an uptick in referrals from general pediatricians, which has since tapered off.

“It’s probably like for all of us: We read an article, it’s fresh on the mind, then you forget about the article and what you’ve read. So we need a little reinforcement from a learning perspective. This is a great article,” he said.

The guidelines debunk as myth the classic teaching that infantile hemangiomas go away. Explicit information is provided about the high-risk anatomic sites warranting consideration for early referral, including the periocular, lumbosacral, and perineal areas, the lip, and lower face.

“The major point is early identification of those lesions requiring evaluation and intervention. Hemangiomas generally speaking are at their ultimate size by 3-5 months of age. The bottom line is if you think something needs to be done, please send that patient, or act upon that patient, sooner rather than later. I can’t tell you how many cases of hemangiomas I’ve seen when the kid is 18 months of age, 3 years of age, 5 years, with a large area of redundant skin, scarring, or something of that sort, and it would have been really nice to have seen them earlier and acted upon them then,” the pediatric dermatologist said.

The guidelines recommend intervention or referral by 1 month of age, ideally. Guidance is provided about the use of oral propranolol as first-line therapy.

“Propranolol is something that has been a real game changer for us,” he noted. “Many people continue to be worried about side effects in using this, particularly in the young childhood population, but this paper shows pretty clearly that hypotension or bradycardia is not a real concern. I never hospitalize these patients for propranolol therapy except in high-risk populations: very preemie, any history of breathing problems. We check the blood pressure and heart rate at baseline, again at 7-10 days, and at every visit. We’ve never found any significant drop in blood pressure.”

Dr. Levy reported financial relationships with half a dozen pharmaceutical companies, none relevant to his presentation.

SDEF/Global Academy for Medical Education and this news organization are owned by the same parent company.

[email protected]

LAHAINA, HAWAII – Reassuring evidence of the safety of oral propranolol for treatment of complicated infantile hemangiomas in patients with PHACE syndrome comes from a recent multicenter study.

Bruce Jancin/MDedge News

Oral propranolol is now well-ensconced as first-line therapy for complicated infantile hemangiomas in otherwise healthy children. However, the beta-blocker’s use in PHACE (Posterior fossa malformations, Hemangiomas, Arterial anomalies, Cardiac defects, and Eye abnormalities) syndrome has been controversial, with concerns raised by some that it might raise the risk for arterial ischemic stroke. Not so, Moise L. Levy, MD, said at the Hawaii Dermatology Seminar provided by Global Academy for Medical Education/Skin Disease Education Foundation.

“I’m not suggesting you use propranolol with reckless abandon in this population, but this stroke concern is something that should be put to bed based on this study,” advised Dr. Levy, professor of dermatology and pediatrics at Dell Medical School in Austin, Tex., and physician-in-chief at Dell Children’s Medical Center.

PHACE syndrome is characterized by large, thick, plaque-like hemangiomas greater than 5 cm in size, most commonly on the face, although they can be located elsewhere.

“There was concern that if you found severely altered cerebrovascular arterial flow and you put a kid on a beta-blocker you might be causing some harm. But what I will tell you is that in this recently published paper this was not in fact an issue,” he said.

Dr. Levy was not an investigator in the multicenter retrospective study, which included 76 patients with PHACE syndrome treated for infantile hemangioma with oral propranolol at 0.3 mg/kg per dose or more at 11 academic tertiary care pediatric dermatology clinics. Treatment started at a median age of 56 days.

There were no strokes, TIAs, cardiovascular events, or other significant problems associated with treatment. Twenty-nine children experienced mild adverse events: minor gastrointestinal or respiratory symptoms, and sleep disturbances were threefold more frequent than reported with placebo in another study. The investigators noted that the safety experience in their PHACE syndrome population compared favorably with that in 726 infants without PHACE syndrome who received oral propranolol for hemangiomas, where the incidence of serious adverse events on treatment was 0.4% (JAMA Dermatol. 2019 Dec 11. doi: 10.1001/jamadermatol.2019.3839).

 

‘Hemangiomas – but we were taught that they go away’

Dr. Levy gave a shout-out to the American Academy of Pediatrics for publishing interdisciplinary expert consensus-based practice guidelines for the management of infantile hemangiomas, which he praised as “quite well done” (Pediatrics. 2019 Jan;143[1]. pii: e20183475. doi: 10.1542/peds.2018-3475).

Following release of the guidelines last year, he and other pediatric vascular anomalies experts saw an uptick in referrals from general pediatricians, which has since tapered off.

“It’s probably like for all of us: We read an article, it’s fresh on the mind, then you forget about the article and what you’ve read. So we need a little reinforcement from a learning perspective. This is a great article,” he said.

The guidelines debunk as myth the classic teaching that infantile hemangiomas go away. Explicit information is provided about the high-risk anatomic sites warranting consideration for early referral, including the periocular, lumbosacral, and perineal areas, the lip, and lower face.

“The major point is early identification of those lesions requiring evaluation and intervention. Hemangiomas generally speaking are at their ultimate size by 3-5 months of age. The bottom line is if you think something needs to be done, please send that patient, or act upon that patient, sooner rather than later. I can’t tell you how many cases of hemangiomas I’ve seen when the kid is 18 months of age, 3 years of age, 5 years, with a large area of redundant skin, scarring, or something of that sort, and it would have been really nice to have seen them earlier and acted upon them then,” the pediatric dermatologist said.

The guidelines recommend intervention or referral by 1 month of age, ideally. Guidance is provided about the use of oral propranolol as first-line therapy.

“Propranolol is something that has been a real game changer for us,” he noted. “Many people continue to be worried about side effects in using this, particularly in the young childhood population, but this paper shows pretty clearly that hypotension or bradycardia is not a real concern. I never hospitalize these patients for propranolol therapy except in high-risk populations: very preemie, any history of breathing problems. We check the blood pressure and heart rate at baseline, again at 7-10 days, and at every visit. We’ve never found any significant drop in blood pressure.”

Dr. Levy reported financial relationships with half a dozen pharmaceutical companies, none relevant to his presentation.

SDEF/Global Academy for Medical Education and this news organization are owned by the same parent company.

[email protected]

To the Editor:

Acanthosis nigricans (AN) is characterized by asymptomatic, hyperpigmented, velvety plaques that can occur anywhere on the body but most often present on the skin of the neck, axillae, groin, and other body folds.^1-12 Although there are 5 subtypes, benign AN is the most common and is related to insulin resistance.^1-4 Insulin can bind to insulinlike growth factor 1 (IGF-1) on keratinocytes, stimulating their proliferation. In type 2 diabetes mellitus, endogenous insulin levels are high enough to bind IGF-1 and activate keratinocytes, leading to the development of AN. Insulin injections have been associated with cutaneous side effects including lipoatrophy, lipohypertrophy, and postinflammatory hyperpigmentation.³ Acanthosis nigricans at insulin injection sites is a rare clinical condition.

A 64-year-old man presented for evaluation of a growing asymptomatic lesion on the abdomen of 6 years’ duration. He had a 17-year history of type 2 diabetes mellitus treated with insulin injections for 14 years after failing oral hypoglycemic agents. The patient reported injecting at the same site on the abdomen for the last 14 years. Physical examination revealed a lichenified, hyperpigmented, verrucous plaque on the lower abdomen under the umbilicus (Figure 1). No similar skin lesions were observed elsewhere on the body. A punch biopsy of the plaque showed hyperkeratosis, papillomatosis, acanthosis, and hyperpigmentation, which was consistent with AN (Figure 2). The patient was instructed to rotate injection sites and avoid the affected area. Over-the-counter ammonium lactate cream applied twice daily to the affected site also was recommended. After 2 months of treatment with this regimen, the patient reported softening and lightening of the lesion on the abdomen.

A PubMed search of articles indexed for MEDLINE for all English-language studies with human participants using the terms acanthosis nigricans and insulin injections yielded 20 results. Of them, 13 discussed localized AN at insulin injection sites: 12 case reports (Table)^1-12 and 1 observational study in a group of diabetic patients.¹³

It has been suggested that localized AN at insulin injection sites develops through similar mechanisms as benign AN. Contributing factors to the development of benign AN may be IGF-1, fibroblast growth factor, and epidermal growth factor.^1-3 Insulin is similar in structure to IGF-1 and can bind to IGF-1 receptors at high enough concentrations. Insulinlike growth factor 1 receptors are present on keratinocytes and fibroblasts, which proliferate upon activation, leading to the development of AN.^1-3 Localized AN is thought to occur when repetitive insulin at the injection site saturates IGF-1 receptors on local keratinocytes.

Based on our patient and prior reports in the literature, localized AN is an uncommon cutaneous complication of insulin injections. Physicians should ask about repetitive insulin injections in the same site when localized AN occurs in patients with diabetes mellitus on insulin therapy. They also should discuss the importance of rotating sites for insulin adminstration to prevent the development of cutaneous complications including AN.

To the Editor:

Acanthosis nigricans (AN) is characterized by asymptomatic, hyperpigmented, velvety plaques that can occur anywhere on the body but most often present on the skin of the neck, axillae, groin, and other body folds.^1-12 Although there are 5 subtypes, benign AN is the most common and is related to insulin resistance.^1-4 Insulin can bind to insulinlike growth factor 1 (IGF-1) on keratinocytes, stimulating their proliferation. In type 2 diabetes mellitus, endogenous insulin levels are high enough to bind IGF-1 and activate keratinocytes, leading to the development of AN. Insulin injections have been associated with cutaneous side effects including lipoatrophy, lipohypertrophy, and postinflammatory hyperpigmentation.³ Acanthosis nigricans at insulin injection sites is a rare clinical condition.

A 64-year-old man presented for evaluation of a growing asymptomatic lesion on the abdomen of 6 years’ duration. He had a 17-year history of type 2 diabetes mellitus treated with insulin injections for 14 years after failing oral hypoglycemic agents. The patient reported injecting at the same site on the abdomen for the last 14 years. Physical examination revealed a lichenified, hyperpigmented, verrucous plaque on the lower abdomen under the umbilicus (Figure 1). No similar skin lesions were observed elsewhere on the body. A punch biopsy of the plaque showed hyperkeratosis, papillomatosis, acanthosis, and hyperpigmentation, which was consistent with AN (Figure 2). The patient was instructed to rotate injection sites and avoid the affected area. Over-the-counter ammonium lactate cream applied twice daily to the affected site also was recommended. After 2 months of treatment with this regimen, the patient reported softening and lightening of the lesion on the abdomen.

A PubMed search of articles indexed for MEDLINE for all English-language studies with human participants using the terms acanthosis nigricans and insulin injections yielded 20 results. Of them, 13 discussed localized AN at insulin injection sites: 12 case reports (Table)^1-12 and 1 observational study in a group of diabetic patients.¹³

It has been suggested that localized AN at insulin injection sites develops through similar mechanisms as benign AN. Contributing factors to the development of benign AN may be IGF-1, fibroblast growth factor, and epidermal growth factor.^1-3 Insulin is similar in structure to IGF-1 and can bind to IGF-1 receptors at high enough concentrations. Insulinlike growth factor 1 receptors are present on keratinocytes and fibroblasts, which proliferate upon activation, leading to the development of AN.^1-3 Localized AN is thought to occur when repetitive insulin at the injection site saturates IGF-1 receptors on local keratinocytes.

Based on our patient and prior reports in the literature, localized AN is an uncommon cutaneous complication of insulin injections. Physicians should ask about repetitive insulin injections in the same site when localized AN occurs in patients with diabetes mellitus on insulin therapy. They also should discuss the importance of rotating sites for insulin adminstration to prevent the development of cutaneous complications including AN.

To the Editor:

Acanthosis nigricans (AN) is characterized by asymptomatic, hyperpigmented, velvety plaques that can occur anywhere on the body but most often present on the skin of the neck, axillae, groin, and other body folds.^1-12 Although there are 5 subtypes, benign AN is the most common and is related to insulin resistance.^1-4 Insulin can bind to insulinlike growth factor 1 (IGF-1) on keratinocytes, stimulating their proliferation. In type 2 diabetes mellitus, endogenous insulin levels are high enough to bind IGF-1 and activate keratinocytes, leading to the development of AN. Insulin injections have been associated with cutaneous side effects including lipoatrophy, lipohypertrophy, and postinflammatory hyperpigmentation.³ Acanthosis nigricans at insulin injection sites is a rare clinical condition.

A 64-year-old man presented for evaluation of a growing asymptomatic lesion on the abdomen of 6 years’ duration. He had a 17-year history of type 2 diabetes mellitus treated with insulin injections for 14 years after failing oral hypoglycemic agents. The patient reported injecting at the same site on the abdomen for the last 14 years. Physical examination revealed a lichenified, hyperpigmented, verrucous plaque on the lower abdomen under the umbilicus (Figure 1). No similar skin lesions were observed elsewhere on the body. A punch biopsy of the plaque showed hyperkeratosis, papillomatosis, acanthosis, and hyperpigmentation, which was consistent with AN (Figure 2). The patient was instructed to rotate injection sites and avoid the affected area. Over-the-counter ammonium lactate cream applied twice daily to the affected site also was recommended. After 2 months of treatment with this regimen, the patient reported softening and lightening of the lesion on the abdomen.

A PubMed search of articles indexed for MEDLINE for all English-language studies with human participants using the terms acanthosis nigricans and insulin injections yielded 20 results. Of them, 13 discussed localized AN at insulin injection sites: 12 case reports (Table)^1-12 and 1 observational study in a group of diabetic patients.¹³

It has been suggested that localized AN at insulin injection sites develops through similar mechanisms as benign AN. Contributing factors to the development of benign AN may be IGF-1, fibroblast growth factor, and epidermal growth factor.^1-3 Insulin is similar in structure to IGF-1 and can bind to IGF-1 receptors at high enough concentrations. Insulinlike growth factor 1 receptors are present on keratinocytes and fibroblasts, which proliferate upon activation, leading to the development of AN.^1-3 Localized AN is thought to occur when repetitive insulin at the injection site saturates IGF-1 receptors on local keratinocytes.

Based on our patient and prior reports in the literature, localized AN is an uncommon cutaneous complication of insulin injections. Physicians should ask about repetitive insulin injections in the same site when localized AN occurs in patients with diabetes mellitus on insulin therapy. They also should discuss the importance of rotating sites for insulin adminstration to prevent the development of cutaneous complications including AN.

The Diagnosis: Incontinentia Pigmenti 

The patient was diagnosed clinically with the vesicular stage of incontinentia pigmenti (IP), a rare, X-linked dominant neuroectodermal dysplasia that usually is lethal in males. The genetic mutation has been identified in the IKBKG gene (inhibitor of nuclear factor κB; formally NEMO), which leads to a truncated and defective nuclear factor κB. Female infants survive and display characteristic findings on examination due to X-inactivation leading to mosaicism.¹ Worldwide, there are approximately 27.6 new cases of IP per year. Although it is heritable, the majority (65%-75%) of cases are due to sporadic mutations, with the remaining minority (25%-35%) representing familial disease.¹ 

Cutaneous findings of IP classically progress through 4 stages, though individual patients often do not develop the characteristic lesions of each of the 4 stages. The vesicular stage (stage 1) presented in our patient (quiz image). This stage presents within 2 weeks of birth in 90% of patients and typically disappears when the patient is approximately 4 months of age.^1-3 Although the clinical presentation is striking, it is essential to rule out herpes simplex virus infection, which can mimic vesicular IP. Localized herpes simplex virus is most commonly seen in clusters on the scalp and often is not present at birth. Alternatively, IP is most often seen on the extremities in bands or whorls of distribution along Blaschko lines,⁴ as in this patient. 

Stage 2 (the verrucous stage) presents with verrucous papules or pustules in a similar blaschkoid distribution. Areas previously involved in stage 1 are not always the same areas affected in stage 2. Approximately 70% of patients develop stage 2 lesions, usually at 2 to 6 weeks of age.^1-3 Erythema toxicum neonatorum presents in the first week of life with pustules often on the trunk or extremities, but these lesions are not confined to Blaschko lines, differentiating it from IP.⁴ 

The third stage (hyperpigmented stage) lends the disease its name and occurs in 90% to 95% of patients with IP. Linear and whorled hyperpigmentation develops in early infancy and can either persist or fade by adolescence.¹ Pustules and hyperpigmentation in transient neonatal pustular melanosis may be similar to this stage of IP, but the distribution is more variable and progression to other lesions is not seen.⁵ 

The fourth and final stage is the hypopigmented stage, whereby blaschkoid linear and whorled lines of hypopigmentation with or without both atrophy and alopecia develop in 75% of patients. This is the last finding, beginning in adolescence and often persisting into adulthood.¹ Goltz syndrome is another X-linked dominant disorder with features similar to IP. Verrucous and atrophic lesions along Blaschko lines are reminiscent of the second and fourth stages of IP but are differentiated in Goltz syndrome because they present concurrently rather than in sequential stages such as IP. Similar extracutaneous organs are affected such as the eyes, teeth, and nails; however, Goltz syndrome may be associated with more distinguishing systemic signs such as sweating and skeletal abnormalities.⁶ 

Given its unique appearance, physicians usually diagnose IP clinically after identification of characteristic linear lesions along the lines of Blaschko in an infant or neonate. Skin biopsy is confirmatory, which would differ depending on the stage of disease biopsied. The vesicular stage is characterized by eosinophilic spongiosis and is differentiated from other items on the histologic differential diagnosis by the presence of dyskeratosis.⁷ Genetic testing is available and should be performed along with a physical examination of the mother for counseling  purposes.¹ 

Proper diagnosis is critical because of the potential multisystem nature of the disease with implications for longitudinal care and prognosis in patients. As in other neurocutaneous disease, IP can affect the hair, nails, teeth, central nervous system, and eyes. All IP patients receive a referral to ophthalmology at the time of diagnosis for a dilated fundus examination, with repeat examinations every several months initially--every 3 months for a year, every 6 months from 1 to 3 years of age--and annually thereafter. Dental evaluation should occur at 6 months of age or whenever the first tooth erupts.¹ Mental retardation, seizures, and developmental delay can occur and usually are evident in the first year of life. Patients should have developmental milestones closely  monitored and be referred to appropriate specialists if signs or symptoms develop consistent with neurologic involvement.¹ 

The Diagnosis: Incontinentia Pigmenti 

The patient was diagnosed clinically with the vesicular stage of incontinentia pigmenti (IP), a rare, X-linked dominant neuroectodermal dysplasia that usually is lethal in males. The genetic mutation has been identified in the IKBKG gene (inhibitor of nuclear factor κB; formally NEMO), which leads to a truncated and defective nuclear factor κB. Female infants survive and display characteristic findings on examination due to X-inactivation leading to mosaicism.¹ Worldwide, there are approximately 27.6 new cases of IP per year. Although it is heritable, the majority (65%-75%) of cases are due to sporadic mutations, with the remaining minority (25%-35%) representing familial disease.¹ 

Cutaneous findings of IP classically progress through 4 stages, though individual patients often do not develop the characteristic lesions of each of the 4 stages. The vesicular stage (stage 1) presented in our patient (quiz image). This stage presents within 2 weeks of birth in 90% of patients and typically disappears when the patient is approximately 4 months of age.^1-3 Although the clinical presentation is striking, it is essential to rule out herpes simplex virus infection, which can mimic vesicular IP. Localized herpes simplex virus is most commonly seen in clusters on the scalp and often is not present at birth. Alternatively, IP is most often seen on the extremities in bands or whorls of distribution along Blaschko lines,⁴ as in this patient. 

Stage 2 (the verrucous stage) presents with verrucous papules or pustules in a similar blaschkoid distribution. Areas previously involved in stage 1 are not always the same areas affected in stage 2. Approximately 70% of patients develop stage 2 lesions, usually at 2 to 6 weeks of age.^1-3 Erythema toxicum neonatorum presents in the first week of life with pustules often on the trunk or extremities, but these lesions are not confined to Blaschko lines, differentiating it from IP.⁴ 

The third stage (hyperpigmented stage) lends the disease its name and occurs in 90% to 95% of patients with IP. Linear and whorled hyperpigmentation develops in early infancy and can either persist or fade by adolescence.¹ Pustules and hyperpigmentation in transient neonatal pustular melanosis may be similar to this stage of IP, but the distribution is more variable and progression to other lesions is not seen.⁵ 

The fourth and final stage is the hypopigmented stage, whereby blaschkoid linear and whorled lines of hypopigmentation with or without both atrophy and alopecia develop in 75% of patients. This is the last finding, beginning in adolescence and often persisting into adulthood.¹ Goltz syndrome is another X-linked dominant disorder with features similar to IP. Verrucous and atrophic lesions along Blaschko lines are reminiscent of the second and fourth stages of IP but are differentiated in Goltz syndrome because they present concurrently rather than in sequential stages such as IP. Similar extracutaneous organs are affected such as the eyes, teeth, and nails; however, Goltz syndrome may be associated with more distinguishing systemic signs such as sweating and skeletal abnormalities.⁶ 

Given its unique appearance, physicians usually diagnose IP clinically after identification of characteristic linear lesions along the lines of Blaschko in an infant or neonate. Skin biopsy is confirmatory, which would differ depending on the stage of disease biopsied. The vesicular stage is characterized by eosinophilic spongiosis and is differentiated from other items on the histologic differential diagnosis by the presence of dyskeratosis.⁷ Genetic testing is available and should be performed along with a physical examination of the mother for counseling  purposes.¹ 

Proper diagnosis is critical because of the potential multisystem nature of the disease with implications for longitudinal care and prognosis in patients. As in other neurocutaneous disease, IP can affect the hair, nails, teeth, central nervous system, and eyes. All IP patients receive a referral to ophthalmology at the time of diagnosis for a dilated fundus examination, with repeat examinations every several months initially--every 3 months for a year, every 6 months from 1 to 3 years of age--and annually thereafter. Dental evaluation should occur at 6 months of age or whenever the first tooth erupts.¹ Mental retardation, seizures, and developmental delay can occur and usually are evident in the first year of life. Patients should have developmental milestones closely  monitored and be referred to appropriate specialists if signs or symptoms develop consistent with neurologic involvement.¹ 

The Diagnosis: Incontinentia Pigmenti 

The patient was diagnosed clinically with the vesicular stage of incontinentia pigmenti (IP), a rare, X-linked dominant neuroectodermal dysplasia that usually is lethal in males. The genetic mutation has been identified in the IKBKG gene (inhibitor of nuclear factor κB; formally NEMO), which leads to a truncated and defective nuclear factor κB. Female infants survive and display characteristic findings on examination due to X-inactivation leading to mosaicism.¹ Worldwide, there are approximately 27.6 new cases of IP per year. Although it is heritable, the majority (65%-75%) of cases are due to sporadic mutations, with the remaining minority (25%-35%) representing familial disease.¹ 

Cutaneous findings of IP classically progress through 4 stages, though individual patients often do not develop the characteristic lesions of each of the 4 stages. The vesicular stage (stage 1) presented in our patient (quiz image). This stage presents within 2 weeks of birth in 90% of patients and typically disappears when the patient is approximately 4 months of age.^1-3 Although the clinical presentation is striking, it is essential to rule out herpes simplex virus infection, which can mimic vesicular IP. Localized herpes simplex virus is most commonly seen in clusters on the scalp and often is not present at birth. Alternatively, IP is most often seen on the extremities in bands or whorls of distribution along Blaschko lines,⁴ as in this patient. 

Stage 2 (the verrucous stage) presents with verrucous papules or pustules in a similar blaschkoid distribution. Areas previously involved in stage 1 are not always the same areas affected in stage 2. Approximately 70% of patients develop stage 2 lesions, usually at 2 to 6 weeks of age.^1-3 Erythema toxicum neonatorum presents in the first week of life with pustules often on the trunk or extremities, but these lesions are not confined to Blaschko lines, differentiating it from IP.⁴ 

The third stage (hyperpigmented stage) lends the disease its name and occurs in 90% to 95% of patients with IP. Linear and whorled hyperpigmentation develops in early infancy and can either persist or fade by adolescence.¹ Pustules and hyperpigmentation in transient neonatal pustular melanosis may be similar to this stage of IP, but the distribution is more variable and progression to other lesions is not seen.⁵ 

The fourth and final stage is the hypopigmented stage, whereby blaschkoid linear and whorled lines of hypopigmentation with or without both atrophy and alopecia develop in 75% of patients. This is the last finding, beginning in adolescence and often persisting into adulthood.¹ Goltz syndrome is another X-linked dominant disorder with features similar to IP. Verrucous and atrophic lesions along Blaschko lines are reminiscent of the second and fourth stages of IP but are differentiated in Goltz syndrome because they present concurrently rather than in sequential stages such as IP. Similar extracutaneous organs are affected such as the eyes, teeth, and nails; however, Goltz syndrome may be associated with more distinguishing systemic signs such as sweating and skeletal abnormalities.⁶ 

Given its unique appearance, physicians usually diagnose IP clinically after identification of characteristic linear lesions along the lines of Blaschko in an infant or neonate. Skin biopsy is confirmatory, which would differ depending on the stage of disease biopsied. The vesicular stage is characterized by eosinophilic spongiosis and is differentiated from other items on the histologic differential diagnosis by the presence of dyskeratosis.⁷ Genetic testing is available and should be performed along with a physical examination of the mother for counseling  purposes.¹ 

Proper diagnosis is critical because of the potential multisystem nature of the disease with implications for longitudinal care and prognosis in patients. As in other neurocutaneous disease, IP can affect the hair, nails, teeth, central nervous system, and eyes. All IP patients receive a referral to ophthalmology at the time of diagnosis for a dilated fundus examination, with repeat examinations every several months initially--every 3 months for a year, every 6 months from 1 to 3 years of age--and annually thereafter. Dental evaluation should occur at 6 months of age or whenever the first tooth erupts.¹ Mental retardation, seizures, and developmental delay can occur and usually are evident in the first year of life. Patients should have developmental milestones closely  monitored and be referred to appropriate specialists if signs or symptoms develop consistent with neurologic involvement.¹ 

LAHAINA, HAWAII – Individuals with vitiligo demonstrated a markedly reduced rate of internal malignancies in a recent first-of-its-kind “big data” study from South Korea, Iltefat Hamzavi, MD, said at the Hawaii Dermatology Seminar provided by Global Academy for Medical Education/Skin Disease Education Foundation.

Bruce Jancin/MDedge News

Previous studies by Dr. Hamzavi and others have established that vitiligo patients have a reduced risk of melanoma and perhaps nonmelanoma skin cancers as well. But the South Korean national study of 101,078 vitiligo patients matched by age and sex to twice as many vitiligo-free controls was the first large examination of the association between vitiligo and internal malignancies. The findings suggest that immunosurveillance in patients with the disease is not merely a skin-deep phenomenon, noted Dr. Hamzavi, of the MultiCultural Dermatology Center at Henry Ford Hospital in Detroit.

“Vitiligo is probably a systemic disease in which there may be increased immunosurveillance. The point here is that as we suppress the disease, we have to be careful that we’re not going to increase cancer rates,” the dermatologist explained in an interview. “This is big data, and something to be aware of, but don’t act on it yet in clinical practice. I just want people to be aware that all of these autoimmune diseases are there for a reason. There are lower rates of melanoma and internal cancers in patients who have vitiligo, but what that means for our new therapies that are coming up we don’t know yet.”

He predicted that the study will open up an active new research domain, but it will take time to find definitive answers as to whether emerging immunomodulatory therapies for patients with vitiligo might, in some instances, increase their current favorably lower risk of internal malignancies. In the meantime, physicians interested in treating vitiligo off label with, for example, Janus kinase (JAK) inhibitors will want to be particularly cautious in patients with a strong history of skin cancer or internal malignancies.

The retrospective, population-based study utilized data from the Korean National Health Insurance claims database. The investigators found that the incidence rate of internal malignancies was 612.9 per 100,000 person-years in the vitiligo group and 708.9 per 100,000 person-years in controls, for a statistically significant and clinically meaningful 14% relative risk reduction after adjustment for age, sex, and comorbid conditions.

Among the most striking organ-specific findings: the vitiligo group had a 38% relative risk reduction in colorectal cancer, a 25% reduction in the risk of lung cancer, and a 38% decrease in ovarian cancer. In contrast, they had a 20% increase in the risk of thyroid cancer (J Clin Oncol. 2019 Apr 10;37[11]:903-11).

Despite the fact that vitiligo is a common disease that affects 0.5%-1% of the population worldwide, for decades it has been something of a pharmacotherapeutic backwater. That’s changed recently and in dramatic fashion as a result of new understanding of the disease pathogenesis. The JAK inhibitors are now under active investigation for the treatment of vitiligo. Indeed, ruxolitinib cream, a potent JAK-1 and -2 inhibitor, is now in phase 3 investigation following a highly successful phase 2 trial. Interleukin-15 blockade is another promising avenue.

Dr. Hamzavi reported serving as a consultant to AbbVie, Aclaris, Novartis, and Pfizer, and receiving research funding from Estee Lauder, Clinuvel Pharmaceuticals, Incyte, and Pfizer. SDEF/Global Academy for Medical Education and this news organization are owned by the same parent company.

[email protected]

LAHAINA, HAWAII – Individuals with vitiligo demonstrated a markedly reduced rate of internal malignancies in a recent first-of-its-kind “big data” study from South Korea, Iltefat Hamzavi, MD, said at the Hawaii Dermatology Seminar provided by Global Academy for Medical Education/Skin Disease Education Foundation.

Bruce Jancin/MDedge News

Previous studies by Dr. Hamzavi and others have established that vitiligo patients have a reduced risk of melanoma and perhaps nonmelanoma skin cancers as well. But the South Korean national study of 101,078 vitiligo patients matched by age and sex to twice as many vitiligo-free controls was the first large examination of the association between vitiligo and internal malignancies. The findings suggest that immunosurveillance in patients with the disease is not merely a skin-deep phenomenon, noted Dr. Hamzavi, of the MultiCultural Dermatology Center at Henry Ford Hospital in Detroit.

“Vitiligo is probably a systemic disease in which there may be increased immunosurveillance. The point here is that as we suppress the disease, we have to be careful that we’re not going to increase cancer rates,” the dermatologist explained in an interview. “This is big data, and something to be aware of, but don’t act on it yet in clinical practice. I just want people to be aware that all of these autoimmune diseases are there for a reason. There are lower rates of melanoma and internal cancers in patients who have vitiligo, but what that means for our new therapies that are coming up we don’t know yet.”

He predicted that the study will open up an active new research domain, but it will take time to find definitive answers as to whether emerging immunomodulatory therapies for patients with vitiligo might, in some instances, increase their current favorably lower risk of internal malignancies. In the meantime, physicians interested in treating vitiligo off label with, for example, Janus kinase (JAK) inhibitors will want to be particularly cautious in patients with a strong history of skin cancer or internal malignancies.

The retrospective, population-based study utilized data from the Korean National Health Insurance claims database. The investigators found that the incidence rate of internal malignancies was 612.9 per 100,000 person-years in the vitiligo group and 708.9 per 100,000 person-years in controls, for a statistically significant and clinically meaningful 14% relative risk reduction after adjustment for age, sex, and comorbid conditions.

Among the most striking organ-specific findings: the vitiligo group had a 38% relative risk reduction in colorectal cancer, a 25% reduction in the risk of lung cancer, and a 38% decrease in ovarian cancer. In contrast, they had a 20% increase in the risk of thyroid cancer (J Clin Oncol. 2019 Apr 10;37[11]:903-11).

Despite the fact that vitiligo is a common disease that affects 0.5%-1% of the population worldwide, for decades it has been something of a pharmacotherapeutic backwater. That’s changed recently and in dramatic fashion as a result of new understanding of the disease pathogenesis. The JAK inhibitors are now under active investigation for the treatment of vitiligo. Indeed, ruxolitinib cream, a potent JAK-1 and -2 inhibitor, is now in phase 3 investigation following a highly successful phase 2 trial. Interleukin-15 blockade is another promising avenue.

Dr. Hamzavi reported serving as a consultant to AbbVie, Aclaris, Novartis, and Pfizer, and receiving research funding from Estee Lauder, Clinuvel Pharmaceuticals, Incyte, and Pfizer. SDEF/Global Academy for Medical Education and this news organization are owned by the same parent company.

[email protected]

LAHAINA, HAWAII – Individuals with vitiligo demonstrated a markedly reduced rate of internal malignancies in a recent first-of-its-kind “big data” study from South Korea, Iltefat Hamzavi, MD, said at the Hawaii Dermatology Seminar provided by Global Academy for Medical Education/Skin Disease Education Foundation.

Bruce Jancin/MDedge News

Previous studies by Dr. Hamzavi and others have established that vitiligo patients have a reduced risk of melanoma and perhaps nonmelanoma skin cancers as well. But the South Korean national study of 101,078 vitiligo patients matched by age and sex to twice as many vitiligo-free controls was the first large examination of the association between vitiligo and internal malignancies. The findings suggest that immunosurveillance in patients with the disease is not merely a skin-deep phenomenon, noted Dr. Hamzavi, of the MultiCultural Dermatology Center at Henry Ford Hospital in Detroit.

“Vitiligo is probably a systemic disease in which there may be increased immunosurveillance. The point here is that as we suppress the disease, we have to be careful that we’re not going to increase cancer rates,” the dermatologist explained in an interview. “This is big data, and something to be aware of, but don’t act on it yet in clinical practice. I just want people to be aware that all of these autoimmune diseases are there for a reason. There are lower rates of melanoma and internal cancers in patients who have vitiligo, but what that means for our new therapies that are coming up we don’t know yet.”

He predicted that the study will open up an active new research domain, but it will take time to find definitive answers as to whether emerging immunomodulatory therapies for patients with vitiligo might, in some instances, increase their current favorably lower risk of internal malignancies. In the meantime, physicians interested in treating vitiligo off label with, for example, Janus kinase (JAK) inhibitors will want to be particularly cautious in patients with a strong history of skin cancer or internal malignancies.

The retrospective, population-based study utilized data from the Korean National Health Insurance claims database. The investigators found that the incidence rate of internal malignancies was 612.9 per 100,000 person-years in the vitiligo group and 708.9 per 100,000 person-years in controls, for a statistically significant and clinically meaningful 14% relative risk reduction after adjustment for age, sex, and comorbid conditions.

Among the most striking organ-specific findings: the vitiligo group had a 38% relative risk reduction in colorectal cancer, a 25% reduction in the risk of lung cancer, and a 38% decrease in ovarian cancer. In contrast, they had a 20% increase in the risk of thyroid cancer (J Clin Oncol. 2019 Apr 10;37[11]:903-11).

Despite the fact that vitiligo is a common disease that affects 0.5%-1% of the population worldwide, for decades it has been something of a pharmacotherapeutic backwater. That’s changed recently and in dramatic fashion as a result of new understanding of the disease pathogenesis. The JAK inhibitors are now under active investigation for the treatment of vitiligo. Indeed, ruxolitinib cream, a potent JAK-1 and -2 inhibitor, is now in phase 3 investigation following a highly successful phase 2 trial. Interleukin-15 blockade is another promising avenue.

Dr. Hamzavi reported serving as a consultant to AbbVie, Aclaris, Novartis, and Pfizer, and receiving research funding from Estee Lauder, Clinuvel Pharmaceuticals, Incyte, and Pfizer. SDEF/Global Academy for Medical Education and this news organization are owned by the same parent company.

[email protected]

Fitzpatrick skin type (FST) is the most commonly used classification system in dermatologic practice. It was developed by Thomas B. Fitzpatrick, MD, PhD, in 1975 to assess the propensity of the skin to burn during phototherapy.¹ Fitzpatrick skin type also can be used to assess the clinical benefits and efficacy of cosmetic procedures, including laser hair removal, chemical peel and dermabrasion, tattoo removal, spray tanning, and laser resurfacing for acne scarring.² The original FST classifications included skin types I through IV; skin types V and VI were later added to include individuals of Asian, Indian, and African origin.¹ As a result, FST often is used by providers as a means of describing constitutive skin color and ethnicity.³

How did FST transition from describing the propensity of the skin to burn from UV light exposure to categorizing skin color, thereby becoming a proxy for race? It most likely occurred because there has not been another widely adopted classification system for describing skin color that can be applied to all skin types. Even when the FST classification scale is used as intended, there are inconsistencies with its accuracy; for example, self-reported FSTs have correlated poorly with sunburn risk as well as physician-reported FSTs.^4,5 Although physician-reported FSTs have been demonstrated to correlate with race, race does not consistently correlate with objective measures of pigmentation or self-reported FSTs.⁵ For example, Japanese women often self-identify as FST type II, but Asian skin generally is considered to be nonwhite.¹ Fitzpatrick himself acknowledged that race and ethnicity are cultural and political terms with no scientific basis.⁶ Fitzpatrick skin type also has been demonstrated to correlate poorly with constitutive skin color and minimal erythema dose values.⁷

We conducted an anonymous survey of dermatologists and dermatology trainees to evaluate how providers use FST in their clinical practice as well as how it is used to describe race and ethnicity.

Methods

The survey was distributed electronically to dermatologists and dermatology trainees from March 13 to March 28, 2019, using the Association of Professors of Dermatology listserv, as well as in person at the annual Skin of Color Society meeting in Washington, DC, on February 28, 2019. The 8-item survey included questions about physician demographics (ie, primary practice setting, board certification, and geographic location); whether the respondent identified as an individual with skin of color; and how the respondent utilized FST in clinical notes (ie, describing race/ethnicity, skin cancer risk, and constitutive [baseline] skin color; determining initial phototherapy dosage and suitability for laser treatments, and likelihood of skin burning). A t test was used to determine whether dermatologists who identified as having skin of color utilized FST differently.

Results

A total of 141 surveys were returned, and 140 respondents were included in the final analysis. Given the methods used to distribute the survey, a response rate could not be calculated. The respondents included more board-certified dermatologists (70%) than dermatology trainees (30%). Ninety-three percent of respondents indicated an academic institution as their primary practice location. Notably, 26% of respondents self-identified as having skin of color.

Forty-one percent of all respondents agreed that FST should be included in their clinical documentation. In response to the question “In what scenarios would you refer to FST in a clinical note?” 31% said they used FST to describe patients’ race or ethnicity, 47% used it to describe patients’ constitutive skin color, and 22% utilized it in both scenarios. Respondents who did not identify as having skin of color were more likely to use FST to describe constitutive skin color, though this finding was not statistically significant (P=.063). Anecdotally, providers also included FST in clinical notes on postinflammatory hyperpigmentation, melasma, and treatment with cryotherapy.

Comment

The US Census Bureau has estimated that half of the US population will be of non-European descent by 2050.⁸ As racial and ethnic distinctions continue to be blurred, attempts to include all nonwhite skin types under the umbrella term skin of color becomes increasingly problematic. The true number of skin colors is unknown but likely is infinite, as Brazilian artist Angélica Dass has demonstrated with her photographic project “Humanae” (Figure). Given this shift in demographics and the limitations of the FST, alternative methods of describing skin color must be developed.

The results of our survey suggest that approximately one-third to half of academic dermatologists/dermatology trainees use FST to describe race/ethnicity and/or constitutive skin color. This misuse of FST may occur more frequently among physicians who do not identify as having skin of color. Additionally, misuse of FST in academic settings may be problematic and confusing for medical students who may learn to use this common dermatologic tool outside of its original intent.

We acknowledge that the conundrum of how to classify individuals with nonwhite skin or skin of color is not simply answered. Several alternative skin classification models have been proposed to improve the sensitivity and specificity of identifying patients with skin of color (Table). Refining FST classification is one approach. Employing terms such as skin irritation, tenderness, itching, or skin becoming darker from sun exposure rather than painful burn or tanning may result in better identification.^1,4 A study conducted in India modified the FST questionnaire to acknowledge cultural behaviors.¹⁵ Because lighter skin is culturally valued in this population, patient experience with purposeful sun exposure was limited; thus, the questionnaire was modified to remove questions on the use of tanning booths and/or creams as well as sun exposure and instead included more objective questions regarding dark brown eye color, black and dark brown hair color, and dark brown skin color.¹⁵ Other studies have suggested that patient-reported photosensitivity assessed via a questionnaire is a valid measure for assessing FST but is associated with an overestimation of skin color, known as “the dark shift.”²⁰

Conclusion

Other investigators have criticized the various limitations of FST, including physician vs patient assessment, interview vs questionnaire, and phrasing of questions on skin type.²³ Our findings suggest that medical providers should be cognizant of conflating race and ethnicity with FST. Two authors of this report (O.R.W. and J.E.D.) are medical students with skin of color and frequently have observed the addition of FST to the medical records of patients who were not receiving phototherapy as a proxy for race. We believe that more culturally appropriate and clinically relevant methods for describing skin of color need to be developed and, in the interim, the original intent of FST should be emphasized and incorporated in medical school and resident education.

Acknowledgment
The authors thank Adewole Adamson, MD (Austin, Texas), for discussion and feedback.

Fitzpatrick skin type (FST) is the most commonly used classification system in dermatologic practice. It was developed by Thomas B. Fitzpatrick, MD, PhD, in 1975 to assess the propensity of the skin to burn during phototherapy.¹ Fitzpatrick skin type also can be used to assess the clinical benefits and efficacy of cosmetic procedures, including laser hair removal, chemical peel and dermabrasion, tattoo removal, spray tanning, and laser resurfacing for acne scarring.² The original FST classifications included skin types I through IV; skin types V and VI were later added to include individuals of Asian, Indian, and African origin.¹ As a result, FST often is used by providers as a means of describing constitutive skin color and ethnicity.³

How did FST transition from describing the propensity of the skin to burn from UV light exposure to categorizing skin color, thereby becoming a proxy for race? It most likely occurred because there has not been another widely adopted classification system for describing skin color that can be applied to all skin types. Even when the FST classification scale is used as intended, there are inconsistencies with its accuracy; for example, self-reported FSTs have correlated poorly with sunburn risk as well as physician-reported FSTs.^4,5 Although physician-reported FSTs have been demonstrated to correlate with race, race does not consistently correlate with objective measures of pigmentation or self-reported FSTs.⁵ For example, Japanese women often self-identify as FST type II, but Asian skin generally is considered to be nonwhite.¹ Fitzpatrick himself acknowledged that race and ethnicity are cultural and political terms with no scientific basis.⁶ Fitzpatrick skin type also has been demonstrated to correlate poorly with constitutive skin color and minimal erythema dose values.⁷

We conducted an anonymous survey of dermatologists and dermatology trainees to evaluate how providers use FST in their clinical practice as well as how it is used to describe race and ethnicity.

Methods

The survey was distributed electronically to dermatologists and dermatology trainees from March 13 to March 28, 2019, using the Association of Professors of Dermatology listserv, as well as in person at the annual Skin of Color Society meeting in Washington, DC, on February 28, 2019. The 8-item survey included questions about physician demographics (ie, primary practice setting, board certification, and geographic location); whether the respondent identified as an individual with skin of color; and how the respondent utilized FST in clinical notes (ie, describing race/ethnicity, skin cancer risk, and constitutive [baseline] skin color; determining initial phototherapy dosage and suitability for laser treatments, and likelihood of skin burning). A t test was used to determine whether dermatologists who identified as having skin of color utilized FST differently.

Results

A total of 141 surveys were returned, and 140 respondents were included in the final analysis. Given the methods used to distribute the survey, a response rate could not be calculated. The respondents included more board-certified dermatologists (70%) than dermatology trainees (30%). Ninety-three percent of respondents indicated an academic institution as their primary practice location. Notably, 26% of respondents self-identified as having skin of color.

Forty-one percent of all respondents agreed that FST should be included in their clinical documentation. In response to the question “In what scenarios would you refer to FST in a clinical note?” 31% said they used FST to describe patients’ race or ethnicity, 47% used it to describe patients’ constitutive skin color, and 22% utilized it in both scenarios. Respondents who did not identify as having skin of color were more likely to use FST to describe constitutive skin color, though this finding was not statistically significant (P=.063). Anecdotally, providers also included FST in clinical notes on postinflammatory hyperpigmentation, melasma, and treatment with cryotherapy.

Comment

The US Census Bureau has estimated that half of the US population will be of non-European descent by 2050.⁸ As racial and ethnic distinctions continue to be blurred, attempts to include all nonwhite skin types under the umbrella term skin of color becomes increasingly problematic. The true number of skin colors is unknown but likely is infinite, as Brazilian artist Angélica Dass has demonstrated with her photographic project “Humanae” (Figure). Given this shift in demographics and the limitations of the FST, alternative methods of describing skin color must be developed.

The results of our survey suggest that approximately one-third to half of academic dermatologists/dermatology trainees use FST to describe race/ethnicity and/or constitutive skin color. This misuse of FST may occur more frequently among physicians who do not identify as having skin of color. Additionally, misuse of FST in academic settings may be problematic and confusing for medical students who may learn to use this common dermatologic tool outside of its original intent.

We acknowledge that the conundrum of how to classify individuals with nonwhite skin or skin of color is not simply answered. Several alternative skin classification models have been proposed to improve the sensitivity and specificity of identifying patients with skin of color (Table). Refining FST classification is one approach. Employing terms such as skin irritation, tenderness, itching, or skin becoming darker from sun exposure rather than painful burn or tanning may result in better identification.^1,4 A study conducted in India modified the FST questionnaire to acknowledge cultural behaviors.¹⁵ Because lighter skin is culturally valued in this population, patient experience with purposeful sun exposure was limited; thus, the questionnaire was modified to remove questions on the use of tanning booths and/or creams as well as sun exposure and instead included more objective questions regarding dark brown eye color, black and dark brown hair color, and dark brown skin color.¹⁵ Other studies have suggested that patient-reported photosensitivity assessed via a questionnaire is a valid measure for assessing FST but is associated with an overestimation of skin color, known as “the dark shift.”²⁰

Conclusion

Other investigators have criticized the various limitations of FST, including physician vs patient assessment, interview vs questionnaire, and phrasing of questions on skin type.²³ Our findings suggest that medical providers should be cognizant of conflating race and ethnicity with FST. Two authors of this report (O.R.W. and J.E.D.) are medical students with skin of color and frequently have observed the addition of FST to the medical records of patients who were not receiving phototherapy as a proxy for race. We believe that more culturally appropriate and clinically relevant methods for describing skin of color need to be developed and, in the interim, the original intent of FST should be emphasized and incorporated in medical school and resident education.

Acknowledgment
The authors thank Adewole Adamson, MD (Austin, Texas), for discussion and feedback.

Fitzpatrick skin type (FST) is the most commonly used classification system in dermatologic practice. It was developed by Thomas B. Fitzpatrick, MD, PhD, in 1975 to assess the propensity of the skin to burn during phototherapy.¹ Fitzpatrick skin type also can be used to assess the clinical benefits and efficacy of cosmetic procedures, including laser hair removal, chemical peel and dermabrasion, tattoo removal, spray tanning, and laser resurfacing for acne scarring.² The original FST classifications included skin types I through IV; skin types V and VI were later added to include individuals of Asian, Indian, and African origin.¹ As a result, FST often is used by providers as a means of describing constitutive skin color and ethnicity.³

How did FST transition from describing the propensity of the skin to burn from UV light exposure to categorizing skin color, thereby becoming a proxy for race? It most likely occurred because there has not been another widely adopted classification system for describing skin color that can be applied to all skin types. Even when the FST classification scale is used as intended, there are inconsistencies with its accuracy; for example, self-reported FSTs have correlated poorly with sunburn risk as well as physician-reported FSTs.^4,5 Although physician-reported FSTs have been demonstrated to correlate with race, race does not consistently correlate with objective measures of pigmentation or self-reported FSTs.⁵ For example, Japanese women often self-identify as FST type II, but Asian skin generally is considered to be nonwhite.¹ Fitzpatrick himself acknowledged that race and ethnicity are cultural and political terms with no scientific basis.⁶ Fitzpatrick skin type also has been demonstrated to correlate poorly with constitutive skin color and minimal erythema dose values.⁷

We conducted an anonymous survey of dermatologists and dermatology trainees to evaluate how providers use FST in their clinical practice as well as how it is used to describe race and ethnicity.

Methods

The survey was distributed electronically to dermatologists and dermatology trainees from March 13 to March 28, 2019, using the Association of Professors of Dermatology listserv, as well as in person at the annual Skin of Color Society meeting in Washington, DC, on February 28, 2019. The 8-item survey included questions about physician demographics (ie, primary practice setting, board certification, and geographic location); whether the respondent identified as an individual with skin of color; and how the respondent utilized FST in clinical notes (ie, describing race/ethnicity, skin cancer risk, and constitutive [baseline] skin color; determining initial phototherapy dosage and suitability for laser treatments, and likelihood of skin burning). A t test was used to determine whether dermatologists who identified as having skin of color utilized FST differently.

Results

A total of 141 surveys were returned, and 140 respondents were included in the final analysis. Given the methods used to distribute the survey, a response rate could not be calculated. The respondents included more board-certified dermatologists (70%) than dermatology trainees (30%). Ninety-three percent of respondents indicated an academic institution as their primary practice location. Notably, 26% of respondents self-identified as having skin of color.

Forty-one percent of all respondents agreed that FST should be included in their clinical documentation. In response to the question “In what scenarios would you refer to FST in a clinical note?” 31% said they used FST to describe patients’ race or ethnicity, 47% used it to describe patients’ constitutive skin color, and 22% utilized it in both scenarios. Respondents who did not identify as having skin of color were more likely to use FST to describe constitutive skin color, though this finding was not statistically significant (P=.063). Anecdotally, providers also included FST in clinical notes on postinflammatory hyperpigmentation, melasma, and treatment with cryotherapy.

Comment

The US Census Bureau has estimated that half of the US population will be of non-European descent by 2050.⁸ As racial and ethnic distinctions continue to be blurred, attempts to include all nonwhite skin types under the umbrella term skin of color becomes increasingly problematic. The true number of skin colors is unknown but likely is infinite, as Brazilian artist Angélica Dass has demonstrated with her photographic project “Humanae” (Figure). Given this shift in demographics and the limitations of the FST, alternative methods of describing skin color must be developed.

The results of our survey suggest that approximately one-third to half of academic dermatologists/dermatology trainees use FST to describe race/ethnicity and/or constitutive skin color. This misuse of FST may occur more frequently among physicians who do not identify as having skin of color. Additionally, misuse of FST in academic settings may be problematic and confusing for medical students who may learn to use this common dermatologic tool outside of its original intent.

We acknowledge that the conundrum of how to classify individuals with nonwhite skin or skin of color is not simply answered. Several alternative skin classification models have been proposed to improve the sensitivity and specificity of identifying patients with skin of color (Table). Refining FST classification is one approach. Employing terms such as skin irritation, tenderness, itching, or skin becoming darker from sun exposure rather than painful burn or tanning may result in better identification.^1,4 A study conducted in India modified the FST questionnaire to acknowledge cultural behaviors.¹⁵ Because lighter skin is culturally valued in this population, patient experience with purposeful sun exposure was limited; thus, the questionnaire was modified to remove questions on the use of tanning booths and/or creams as well as sun exposure and instead included more objective questions regarding dark brown eye color, black and dark brown hair color, and dark brown skin color.¹⁵ Other studies have suggested that patient-reported photosensitivity assessed via a questionnaire is a valid measure for assessing FST but is associated with an overestimation of skin color, known as “the dark shift.”²⁰

Conclusion

Other investigators have criticized the various limitations of FST, including physician vs patient assessment, interview vs questionnaire, and phrasing of questions on skin type.²³ Our findings suggest that medical providers should be cognizant of conflating race and ethnicity with FST. Two authors of this report (O.R.W. and J.E.D.) are medical students with skin of color and frequently have observed the addition of FST to the medical records of patients who were not receiving phototherapy as a proxy for race. We believe that more culturally appropriate and clinically relevant methods for describing skin of color need to be developed and, in the interim, the original intent of FST should be emphasized and incorporated in medical school and resident education.

Acknowledgment
The authors thank Adewole Adamson, MD (Austin, Texas), for discussion and feedback.

A new line of Barbie dolls unveiled by Mattel earlier this month includes one with vitiligo, much to the delight of clinicians who treat children and adolescents with the condition.

Mattel, Inc.

“When I see young children and adolescents with vitiligo, it is very common for me to feel their emotional suffering from their skin condition,” Seemal R. Desai, MD, a dermatologist at the University of Texas Southwestern Medical Center in Dallas said in an interview. “Kids can be cruel. Name calling, social ostracizing, [and] effects on self-esteem are all things I have seen amongst my patients and their families in their own struggles with vitiligo.”

According to a brand communications representative from toymaker Mattel, which began manufacturing Barbie dolls in 1959, the company worked with a board-certified dermatologist to include a doll with vitiligo in its 2020 “Fashionistas” line. “As we continue to redefine what it means to be a ‘Barbie’ or look like Barbie, offering a doll with vitiligo in our main doll line allows kids to play out even more stories they see in the world around them,” the representative wrote in an email message. Other dolls debuting as part of the lineup include one with no hair, one with a darker skin tone that uses a gold prosthetic limb, and a Ken doll with long rooted hair (think Jeff Spicoli in “Fast Times at Ridgemont High,” but about six inches longer).

Such efforts to celebrate diversity and inclusiveness go far in helping children and young adults to embrace their skin and their own identities, said Dr. Desai, the immediate past president of the Skin of Color Society and a member of the American Academy of Dermatology board of directors. “One nuance, perhaps even more important, is that the Barbie can help to break down barriers, create awareness, and potentially even reduce bullying, stigma, and lack of knowledge about vitiligo amongst the general public who don’t understand vitiligo,” he said. “I hope the public and social media will embrace this new Barbie. Who knows? Pretty soon, vitiligo may no longer be a ‘thing’ that causes ‘stares’ and ‘glares.’ ”

Referring to the Barbie with no hair in the new line of dolls, the Mattel statement said, “ if a girl is experiencing hair loss for any reason, she can see herself reflected in the line.”

Mattel, Inc.

In 2019, Mattel introduced a lineup of Barbie dolls reflecting permanent disabilities, including one with a prosthetic limb. For that effort, the company collaborated with then-12-year-old Jordan Reeves, the “Born Just Right” coauthor “who is on a mission to build creative solutions that help kids with disabilities, to create a play experience that is as representative as possible,” the Mattel representative wrote.

[email protected]

A new line of Barbie dolls unveiled by Mattel earlier this month includes one with vitiligo, much to the delight of clinicians who treat children and adolescents with the condition.

Mattel, Inc.

“When I see young children and adolescents with vitiligo, it is very common for me to feel their emotional suffering from their skin condition,” Seemal R. Desai, MD, a dermatologist at the University of Texas Southwestern Medical Center in Dallas said in an interview. “Kids can be cruel. Name calling, social ostracizing, [and] effects on self-esteem are all things I have seen amongst my patients and their families in their own struggles with vitiligo.”

According to a brand communications representative from toymaker Mattel, which began manufacturing Barbie dolls in 1959, the company worked with a board-certified dermatologist to include a doll with vitiligo in its 2020 “Fashionistas” line. “As we continue to redefine what it means to be a ‘Barbie’ or look like Barbie, offering a doll with vitiligo in our main doll line allows kids to play out even more stories they see in the world around them,” the representative wrote in an email message. Other dolls debuting as part of the lineup include one with no hair, one with a darker skin tone that uses a gold prosthetic limb, and a Ken doll with long rooted hair (think Jeff Spicoli in “Fast Times at Ridgemont High,” but about six inches longer).

Such efforts to celebrate diversity and inclusiveness go far in helping children and young adults to embrace their skin and their own identities, said Dr. Desai, the immediate past president of the Skin of Color Society and a member of the American Academy of Dermatology board of directors. “One nuance, perhaps even more important, is that the Barbie can help to break down barriers, create awareness, and potentially even reduce bullying, stigma, and lack of knowledge about vitiligo amongst the general public who don’t understand vitiligo,” he said. “I hope the public and social media will embrace this new Barbie. Who knows? Pretty soon, vitiligo may no longer be a ‘thing’ that causes ‘stares’ and ‘glares.’ ”

Referring to the Barbie with no hair in the new line of dolls, the Mattel statement said, “ if a girl is experiencing hair loss for any reason, she can see herself reflected in the line.”

Mattel, Inc.

In 2019, Mattel introduced a lineup of Barbie dolls reflecting permanent disabilities, including one with a prosthetic limb. For that effort, the company collaborated with then-12-year-old Jordan Reeves, the “Born Just Right” coauthor “who is on a mission to build creative solutions that help kids with disabilities, to create a play experience that is as representative as possible,” the Mattel representative wrote.

[email protected]

A new line of Barbie dolls unveiled by Mattel earlier this month includes one with vitiligo, much to the delight of clinicians who treat children and adolescents with the condition.

Mattel, Inc.

“When I see young children and adolescents with vitiligo, it is very common for me to feel their emotional suffering from their skin condition,” Seemal R. Desai, MD, a dermatologist at the University of Texas Southwestern Medical Center in Dallas said in an interview. “Kids can be cruel. Name calling, social ostracizing, [and] effects on self-esteem are all things I have seen amongst my patients and their families in their own struggles with vitiligo.”

According to a brand communications representative from toymaker Mattel, which began manufacturing Barbie dolls in 1959, the company worked with a board-certified dermatologist to include a doll with vitiligo in its 2020 “Fashionistas” line. “As we continue to redefine what it means to be a ‘Barbie’ or look like Barbie, offering a doll with vitiligo in our main doll line allows kids to play out even more stories they see in the world around them,” the representative wrote in an email message. Other dolls debuting as part of the lineup include one with no hair, one with a darker skin tone that uses a gold prosthetic limb, and a Ken doll with long rooted hair (think Jeff Spicoli in “Fast Times at Ridgemont High,” but about six inches longer).

Such efforts to celebrate diversity and inclusiveness go far in helping children and young adults to embrace their skin and their own identities, said Dr. Desai, the immediate past president of the Skin of Color Society and a member of the American Academy of Dermatology board of directors. “One nuance, perhaps even more important, is that the Barbie can help to break down barriers, create awareness, and potentially even reduce bullying, stigma, and lack of knowledge about vitiligo amongst the general public who don’t understand vitiligo,” he said. “I hope the public and social media will embrace this new Barbie. Who knows? Pretty soon, vitiligo may no longer be a ‘thing’ that causes ‘stares’ and ‘glares.’ ”

Referring to the Barbie with no hair in the new line of dolls, the Mattel statement said, “ if a girl is experiencing hair loss for any reason, she can see herself reflected in the line.”

Mattel, Inc.

In 2019, Mattel introduced a lineup of Barbie dolls reflecting permanent disabilities, including one with a prosthetic limb. For that effort, the company collaborated with then-12-year-old Jordan Reeves, the “Born Just Right” coauthor “who is on a mission to build creative solutions that help kids with disabilities, to create a play experience that is as representative as possible,” the Mattel representative wrote.

[email protected]

A dermatologist-led nonprofit organization has entered into a partnership with the United Nations to achieve global progress towards greater inclusivity for people with albinism.

Courtesy NYDG Foundation

Representatives from the New York–based NYDG Foundation, including dermatologist David Colbert, MD, recently signed the agreement with the United Nations High Commissioner for Human Rights. At the center of the inclusivity efforts is the foundation’s ColorFull campaign, which aims to shape a collective response to the discrimination and violence that individuals with albinism face around the world.

“We really need to build more inclusive and communal health care systems for all. Partnering with the United Nations will help us to reach our goals and build stronger bonds with those health care providers working with one of the most marginalized and vulnerable groups in Africa,” Dr. Colbert said in an interview.

Stylish images of individuals with albinism, including prominent model Diandra Forrest, anchor the ColorFull campaign’s messaging; Ms. Forrest is featured in a video posted by the United Nations in November announcing the joint human rights campaign. Because the consequences of albinism can be deadly serious for affected individuals in many parts of the world, awareness is desperately needed, participants in NYDG’s work and in Standing Voice, another nonprofit that provides resources for people with albinism in East Africa, emphasized in interviews.
 

Striving to do good work

Stephan Bognar, a seasoned leader of international nonprofits, has teamed up with Dr. Colbert, NYDG Foundation’s founding physician, to craft the international campaign to raise awareness of albinism and increase acceptance of those with the condition. “You don’t always have to stand alone to break down the walls of exclusion. The fight for social justice and human rights for persons with albinism requires a collective responsibility,” Mr. Bognar said in an interview.

Stephanie Sinclair, courtesy NYDG Foundation

Dr. Colbert, senior partner of the New York Dermatology Group, a large Manhattan-based practice, founded the nonprofit when he became involved in wound-care efforts in Haiti following the 2010 earthquake. The foundation has since supported such philanthropic efforts as helping people with albinism, offering scholarships, and raising awareness of the importance of sun protection among youth athletes.

“One day, 3 years ago or so, I was reading the New York Times and I came across this article – it was called ‘The Hunted,’ ” Dr. Colbert recalled. “It was something I knew nothing about. In Eastern Africa, people with albinism are often hunted down for body parts and their lives are at risk” from being hunted and murdered – but also because their body parts are used for witchcraft and magic, he noted.

“I was captivated by that, and I remember I called Stephan, and I said, ‘I have a project for you.’ ” Because of extensive previous work with international nongovernmental organizations and the United Nations, Mr. Bognar, who is now the executive director of the NYDG Foundation, “had the pedigree to make things happen instead of spinning our wheels,” Dr. Colbert said.

Courtesy Dr. David Colbert

Philanthropic work in dermatology

Despite his busy practice, Dr. Colbert said he has found great satisfaction in pursuing philanthropic work. For physicians considering similar efforts, he said that genuine engagement with the issue is critical and global travel isn’t necessary to make a real difference.

“I think that, first, this should be something that you’re interested in and that you have the means to make some impact,” Dr. Colbert said. “Doing something doesn’t need to be a global campaign. You don’t need to have a home run – every little thing counts. Catching one squamous cell cancer on one patient with albinism makes a difference. But if you want to go bigger, you have to look at your community and see who has the resources and who might also be interested” in a cause you’re passionate about.

He added that a busy physician shouldn’t expect to do it all. “You have to find the right partner because we as physicians are taking care of our patients and paying the rent, so taking on a partner who is trained to do that can ... help you achieve what you envision.”

Though the NYDG Foundation has funded trips to Africa and participates in teledermatology there, Dr. Colbert said that the awareness campaign the NYDG is cosponsoring with the United Nations is of fundamental importance as well. “This is a really great example of the positive impact that social media can have on our society – in a good way, instead of a negative or self-serving way,” he said.

“I think that the ColorFull campaign will normalize the idea of people who are living without melanin in their skin. It keeps it out of the realm of ‘Don’t say anything.’ People don’t know what it means, so if we bring out the science, and show successful people who have normal lives, who have children, and we explain what it is, it demystifies it – and everybody wins. ... We’re all just people, no matter how many melanin granules we have.”

Dr. Colbert reported that he has no relevant conflicts of interest.
 

Standing Voice also provides resources in East Africa

The work of other nongovernmental organizations is also making a difference for people in East Africa with albinism.

Standing Voice is a United Kingdom–based nonprofit that provides education and resources that include sunscreen, as well as assessment and treatment of skin conditions for people with albinism in Tanzania and Malawi.

Courtesy Standing Voice

Courtesy Standing Voice

Courtesy Standing Voice

Courtesy Standing Voice

[email protected]

A dermatologist-led nonprofit organization has entered into a partnership with the United Nations to achieve global progress towards greater inclusivity for people with albinism.

Courtesy NYDG Foundation

Representatives from the New York–based NYDG Foundation, including dermatologist David Colbert, MD, recently signed the agreement with the United Nations High Commissioner for Human Rights. At the center of the inclusivity efforts is the foundation’s ColorFull campaign, which aims to shape a collective response to the discrimination and violence that individuals with albinism face around the world.

“We really need to build more inclusive and communal health care systems for all. Partnering with the United Nations will help us to reach our goals and build stronger bonds with those health care providers working with one of the most marginalized and vulnerable groups in Africa,” Dr. Colbert said in an interview.

Stylish images of individuals with albinism, including prominent model Diandra Forrest, anchor the ColorFull campaign’s messaging; Ms. Forrest is featured in a video posted by the United Nations in November announcing the joint human rights campaign. Because the consequences of albinism can be deadly serious for affected individuals in many parts of the world, awareness is desperately needed, participants in NYDG’s work and in Standing Voice, another nonprofit that provides resources for people with albinism in East Africa, emphasized in interviews.
 

Striving to do good work

Stephan Bognar, a seasoned leader of international nonprofits, has teamed up with Dr. Colbert, NYDG Foundation’s founding physician, to craft the international campaign to raise awareness of albinism and increase acceptance of those with the condition. “You don’t always have to stand alone to break down the walls of exclusion. The fight for social justice and human rights for persons with albinism requires a collective responsibility,” Mr. Bognar said in an interview.

Stephanie Sinclair, courtesy NYDG Foundation

Dr. Colbert, senior partner of the New York Dermatology Group, a large Manhattan-based practice, founded the nonprofit when he became involved in wound-care efforts in Haiti following the 2010 earthquake. The foundation has since supported such philanthropic efforts as helping people with albinism, offering scholarships, and raising awareness of the importance of sun protection among youth athletes.

“One day, 3 years ago or so, I was reading the New York Times and I came across this article – it was called ‘The Hunted,’ ” Dr. Colbert recalled. “It was something I knew nothing about. In Eastern Africa, people with albinism are often hunted down for body parts and their lives are at risk” from being hunted and murdered – but also because their body parts are used for witchcraft and magic, he noted.

“I was captivated by that, and I remember I called Stephan, and I said, ‘I have a project for you.’ ” Because of extensive previous work with international nongovernmental organizations and the United Nations, Mr. Bognar, who is now the executive director of the NYDG Foundation, “had the pedigree to make things happen instead of spinning our wheels,” Dr. Colbert said.

Courtesy Dr. David Colbert

Philanthropic work in dermatology

Despite his busy practice, Dr. Colbert said he has found great satisfaction in pursuing philanthropic work. For physicians considering similar efforts, he said that genuine engagement with the issue is critical and global travel isn’t necessary to make a real difference.

“I think that, first, this should be something that you’re interested in and that you have the means to make some impact,” Dr. Colbert said. “Doing something doesn’t need to be a global campaign. You don’t need to have a home run – every little thing counts. Catching one squamous cell cancer on one patient with albinism makes a difference. But if you want to go bigger, you have to look at your community and see who has the resources and who might also be interested” in a cause you’re passionate about.

He added that a busy physician shouldn’t expect to do it all. “You have to find the right partner because we as physicians are taking care of our patients and paying the rent, so taking on a partner who is trained to do that can ... help you achieve what you envision.”

Though the NYDG Foundation has funded trips to Africa and participates in teledermatology there, Dr. Colbert said that the awareness campaign the NYDG is cosponsoring with the United Nations is of fundamental importance as well. “This is a really great example of the positive impact that social media can have on our society – in a good way, instead of a negative or self-serving way,” he said.

“I think that the ColorFull campaign will normalize the idea of people who are living without melanin in their skin. It keeps it out of the realm of ‘Don’t say anything.’ People don’t know what it means, so if we bring out the science, and show successful people who have normal lives, who have children, and we explain what it is, it demystifies it – and everybody wins. ... We’re all just people, no matter how many melanin granules we have.”

Dr. Colbert reported that he has no relevant conflicts of interest.
 

Standing Voice also provides resources in East Africa

The work of other nongovernmental organizations is also making a difference for people in East Africa with albinism.

Standing Voice is a United Kingdom–based nonprofit that provides education and resources that include sunscreen, as well as assessment and treatment of skin conditions for people with albinism in Tanzania and Malawi.

Courtesy Standing Voice

Courtesy Standing Voice

Courtesy Standing Voice

Courtesy Standing Voice

[email protected]

A dermatologist-led nonprofit organization has entered into a partnership with the United Nations to achieve global progress towards greater inclusivity for people with albinism.

Courtesy NYDG Foundation

Representatives from the New York–based NYDG Foundation, including dermatologist David Colbert, MD, recently signed the agreement with the United Nations High Commissioner for Human Rights. At the center of the inclusivity efforts is the foundation’s ColorFull campaign, which aims to shape a collective response to the discrimination and violence that individuals with albinism face around the world.

“We really need to build more inclusive and communal health care systems for all. Partnering with the United Nations will help us to reach our goals and build stronger bonds with those health care providers working with one of the most marginalized and vulnerable groups in Africa,” Dr. Colbert said in an interview.

Stylish images of individuals with albinism, including prominent model Diandra Forrest, anchor the ColorFull campaign’s messaging; Ms. Forrest is featured in a video posted by the United Nations in November announcing the joint human rights campaign. Because the consequences of albinism can be deadly serious for affected individuals in many parts of the world, awareness is desperately needed, participants in NYDG’s work and in Standing Voice, another nonprofit that provides resources for people with albinism in East Africa, emphasized in interviews.
 

Striving to do good work

Stephan Bognar, a seasoned leader of international nonprofits, has teamed up with Dr. Colbert, NYDG Foundation’s founding physician, to craft the international campaign to raise awareness of albinism and increase acceptance of those with the condition. “You don’t always have to stand alone to break down the walls of exclusion. The fight for social justice and human rights for persons with albinism requires a collective responsibility,” Mr. Bognar said in an interview.

Stephanie Sinclair, courtesy NYDG Foundation

Dr. Colbert, senior partner of the New York Dermatology Group, a large Manhattan-based practice, founded the nonprofit when he became involved in wound-care efforts in Haiti following the 2010 earthquake. The foundation has since supported such philanthropic efforts as helping people with albinism, offering scholarships, and raising awareness of the importance of sun protection among youth athletes.

“One day, 3 years ago or so, I was reading the New York Times and I came across this article – it was called ‘The Hunted,’ ” Dr. Colbert recalled. “It was something I knew nothing about. In Eastern Africa, people with albinism are often hunted down for body parts and their lives are at risk” from being hunted and murdered – but also because their body parts are used for witchcraft and magic, he noted.

“I was captivated by that, and I remember I called Stephan, and I said, ‘I have a project for you.’ ” Because of extensive previous work with international nongovernmental organizations and the United Nations, Mr. Bognar, who is now the executive director of the NYDG Foundation, “had the pedigree to make things happen instead of spinning our wheels,” Dr. Colbert said.

Courtesy Dr. David Colbert

Philanthropic work in dermatology

Despite his busy practice, Dr. Colbert said he has found great satisfaction in pursuing philanthropic work. For physicians considering similar efforts, he said that genuine engagement with the issue is critical and global travel isn’t necessary to make a real difference.

“I think that, first, this should be something that you’re interested in and that you have the means to make some impact,” Dr. Colbert said. “Doing something doesn’t need to be a global campaign. You don’t need to have a home run – every little thing counts. Catching one squamous cell cancer on one patient with albinism makes a difference. But if you want to go bigger, you have to look at your community and see who has the resources and who might also be interested” in a cause you’re passionate about.

He added that a busy physician shouldn’t expect to do it all. “You have to find the right partner because we as physicians are taking care of our patients and paying the rent, so taking on a partner who is trained to do that can ... help you achieve what you envision.”

Though the NYDG Foundation has funded trips to Africa and participates in teledermatology there, Dr. Colbert said that the awareness campaign the NYDG is cosponsoring with the United Nations is of fundamental importance as well. “This is a really great example of the positive impact that social media can have on our society – in a good way, instead of a negative or self-serving way,” he said.

“I think that the ColorFull campaign will normalize the idea of people who are living without melanin in their skin. It keeps it out of the realm of ‘Don’t say anything.’ People don’t know what it means, so if we bring out the science, and show successful people who have normal lives, who have children, and we explain what it is, it demystifies it – and everybody wins. ... We’re all just people, no matter how many melanin granules we have.”

Dr. Colbert reported that he has no relevant conflicts of interest.
 

Standing Voice also provides resources in East Africa

The work of other nongovernmental organizations is also making a difference for people in East Africa with albinism.

Standing Voice is a United Kingdom–based nonprofit that provides education and resources that include sunscreen, as well as assessment and treatment of skin conditions for people with albinism in Tanzania and Malawi.

Courtesy Standing Voice

Courtesy Standing Voice

Courtesy Standing Voice

Courtesy Standing Voice

[email protected]

A low-density nonablative laser successfully treated postinflammatory hyperpigmentation (PIH) in a group of patients with darker skin types, Yoon‐Soo Cindy Bae, MD, and colleagues reported.

Among patients treated with the nonablative fractional 1,927 nm laser, there was a mean improvement of about 43% in hyperpigmented areas, and no side effects were reported, wrote Dr. Bae, of the department of dermatology at New York University and the Laser & Skin Surgery Center of New York, and coauthors in Lasers in Surgery and Medicine.

Lasers have not been the first choice for hyperpigmentation in Fitzpatrick skin types IV, V, and VI, they pointed out. More commonly used treatments are hydroquinone and chemical peels that use glycolic acid or salicylic acid. But these are not always ideal options, Dr. Bae said in an interview.

“There are side effects to medical therapy. The drawbacks of medical therapy include compliance issues, risk of skin irritation from the product ... and a risk of hyperpigmentation specifically for hydroquinone. There are also risks to laser therapy, including dyspigmentation and scarring,” she added. “However, the laser we used is a low energy, nonablative type of laser, so the risk of scarring is extremely rare and the dyspigmentation is actually what we are aiming to treat.”

The retrospective study comprised 61 patients with PIH who had received more than one treatment with the low energy fractionated 1,927 nm diode laser between 2013 and 2016. Most were Fitzpatrick type IV (73.8%). The remainder were Type V (16.4%) and Type VI (9.8%). The most common treatment site was the face or cheeks (68.9%), followed by legs (13%), the rest of the cases were unspecified.

Patients had received treatment with the laser with fixed fluence at 5 mJ, fixed spot size of 140 micrometers, depth of 170 micrometers, and 5% coverage. They required several treatments: 15 had two, 14 had three, 16 had four, and the remainder had five or more. Topical treatment data were not collected. Photographs taken before treatment and before the last treatment were evaluated by dermatologists who had not treated the patients. Based on those evaluations, the mean improvement was a statistically significant 43.2%.

There did not, however, appear to be much difference between the treatment groups. The mean improvement among patients with two treatments was 44.5%; three treatments, 44.29%; four treatments, 40.63%; five or more treatments, 43.75%.

Although those with darker skin types tended to have better results, there were no statistically significant differences between the skin-type groups. Among those with Fitzpatrick skin type IV, the mean improvement was 40.39%; skin type V, 47.25%; and skin type VI, 57.92%.

“The fact that there was no correlation between Fitzpatrick skin type … and average percent improvement demonstrates that this laser is a viable treatment option for patients with very dark skin,” the authors wrote. “There were also no significant differences between the average percent improvements for people receiving different numbers of treatments. A trend was observed that favored treating patients with darker skin type; however, this lacked statistical significance. This may have been due to an underpowered study.”

Limitations of the study included the retrospective design and nonstandardization of photographs; “further studies with prospective controlled designs are needed to confirm our findings,” they added.

No funding or disclosure information was provided.

[email protected]

SOURCE: Bae YS et al. Lasers Surg Med. 2019 Oct 29. doi: 10.1002/lsm.23173.

A low-density nonablative laser successfully treated postinflammatory hyperpigmentation (PIH) in a group of patients with darker skin types, Yoon‐Soo Cindy Bae, MD, and colleagues reported.

Among patients treated with the nonablative fractional 1,927 nm laser, there was a mean improvement of about 43% in hyperpigmented areas, and no side effects were reported, wrote Dr. Bae, of the department of dermatology at New York University and the Laser & Skin Surgery Center of New York, and coauthors in Lasers in Surgery and Medicine.

Lasers have not been the first choice for hyperpigmentation in Fitzpatrick skin types IV, V, and VI, they pointed out. More commonly used treatments are hydroquinone and chemical peels that use glycolic acid or salicylic acid. But these are not always ideal options, Dr. Bae said in an interview.

“There are side effects to medical therapy. The drawbacks of medical therapy include compliance issues, risk of skin irritation from the product ... and a risk of hyperpigmentation specifically for hydroquinone. There are also risks to laser therapy, including dyspigmentation and scarring,” she added. “However, the laser we used is a low energy, nonablative type of laser, so the risk of scarring is extremely rare and the dyspigmentation is actually what we are aiming to treat.”

The retrospective study comprised 61 patients with PIH who had received more than one treatment with the low energy fractionated 1,927 nm diode laser between 2013 and 2016. Most were Fitzpatrick type IV (73.8%). The remainder were Type V (16.4%) and Type VI (9.8%). The most common treatment site was the face or cheeks (68.9%), followed by legs (13%), the rest of the cases were unspecified.

Patients had received treatment with the laser with fixed fluence at 5 mJ, fixed spot size of 140 micrometers, depth of 170 micrometers, and 5% coverage. They required several treatments: 15 had two, 14 had three, 16 had four, and the remainder had five or more. Topical treatment data were not collected. Photographs taken before treatment and before the last treatment were evaluated by dermatologists who had not treated the patients. Based on those evaluations, the mean improvement was a statistically significant 43.2%.

There did not, however, appear to be much difference between the treatment groups. The mean improvement among patients with two treatments was 44.5%; three treatments, 44.29%; four treatments, 40.63%; five or more treatments, 43.75%.

Although those with darker skin types tended to have better results, there were no statistically significant differences between the skin-type groups. Among those with Fitzpatrick skin type IV, the mean improvement was 40.39%; skin type V, 47.25%; and skin type VI, 57.92%.

“The fact that there was no correlation between Fitzpatrick skin type … and average percent improvement demonstrates that this laser is a viable treatment option for patients with very dark skin,” the authors wrote. “There were also no significant differences between the average percent improvements for people receiving different numbers of treatments. A trend was observed that favored treating patients with darker skin type; however, this lacked statistical significance. This may have been due to an underpowered study.”

Limitations of the study included the retrospective design and nonstandardization of photographs; “further studies with prospective controlled designs are needed to confirm our findings,” they added.

No funding or disclosure information was provided.

[email protected]

SOURCE: Bae YS et al. Lasers Surg Med. 2019 Oct 29. doi: 10.1002/lsm.23173.

A low-density nonablative laser successfully treated postinflammatory hyperpigmentation (PIH) in a group of patients with darker skin types, Yoon‐Soo Cindy Bae, MD, and colleagues reported.

Among patients treated with the nonablative fractional 1,927 nm laser, there was a mean improvement of about 43% in hyperpigmented areas, and no side effects were reported, wrote Dr. Bae, of the department of dermatology at New York University and the Laser & Skin Surgery Center of New York, and coauthors in Lasers in Surgery and Medicine.

Lasers have not been the first choice for hyperpigmentation in Fitzpatrick skin types IV, V, and VI, they pointed out. More commonly used treatments are hydroquinone and chemical peels that use glycolic acid or salicylic acid. But these are not always ideal options, Dr. Bae said in an interview.

“There are side effects to medical therapy. The drawbacks of medical therapy include compliance issues, risk of skin irritation from the product ... and a risk of hyperpigmentation specifically for hydroquinone. There are also risks to laser therapy, including dyspigmentation and scarring,” she added. “However, the laser we used is a low energy, nonablative type of laser, so the risk of scarring is extremely rare and the dyspigmentation is actually what we are aiming to treat.”

The retrospective study comprised 61 patients with PIH who had received more than one treatment with the low energy fractionated 1,927 nm diode laser between 2013 and 2016. Most were Fitzpatrick type IV (73.8%). The remainder were Type V (16.4%) and Type VI (9.8%). The most common treatment site was the face or cheeks (68.9%), followed by legs (13%), the rest of the cases were unspecified.

Patients had received treatment with the laser with fixed fluence at 5 mJ, fixed spot size of 140 micrometers, depth of 170 micrometers, and 5% coverage. They required several treatments: 15 had two, 14 had three, 16 had four, and the remainder had five or more. Topical treatment data were not collected. Photographs taken before treatment and before the last treatment were evaluated by dermatologists who had not treated the patients. Based on those evaluations, the mean improvement was a statistically significant 43.2%.

There did not, however, appear to be much difference between the treatment groups. The mean improvement among patients with two treatments was 44.5%; three treatments, 44.29%; four treatments, 40.63%; five or more treatments, 43.75%.

Although those with darker skin types tended to have better results, there were no statistically significant differences between the skin-type groups. Among those with Fitzpatrick skin type IV, the mean improvement was 40.39%; skin type V, 47.25%; and skin type VI, 57.92%.

“The fact that there was no correlation between Fitzpatrick skin type … and average percent improvement demonstrates that this laser is a viable treatment option for patients with very dark skin,” the authors wrote. “There were also no significant differences between the average percent improvements for people receiving different numbers of treatments. A trend was observed that favored treating patients with darker skin type; however, this lacked statistical significance. This may have been due to an underpowered study.”

Limitations of the study included the retrospective design and nonstandardization of photographs; “further studies with prospective controlled designs are needed to confirm our findings,” they added.

No funding or disclosure information was provided.

[email protected]

SOURCE: Bae YS et al. Lasers Surg Med. 2019 Oct 29. doi: 10.1002/lsm.23173.