Janellen Smith, MD

To the Editor:

MYH9-related disorder is an autosomal-dominant disorder characterized by macrothrombocytopenia and neutrophil inclusions secondary to defective myosin-9.¹ We describe a case of lower leg hyperpigmentation secondary to hemosiderin deposition from MYH9-related disorder.

A 31-year-old woman with a history of MYH9-related disorder and mixed connective tissue disease presented to the outpatient dermatology clinic with asymptomatic brown patches on the lower legs (Figure) of 10 years’ duration. She also had epistaxis, hearing loss, renal disease, and menorrhagia secondary to MYH9-related disorder. The patient had been started on hydroxychloroquine 2 years earlier by rheumatology for mixed connective tissue disorder. A biopsy was not performed, given the risk of bleeding from thrombocytopenia. Ammonium lactate lotion was recommended for the leg patches. No further interventions were undertaken. At 6-month follow-up, hyperpigmentation on the lower legs was stable. The patient expressed no desire for cosmetic intervention.

Prior to discovery of a common gene, MYH9-related disorder was classified as 4 overlapping syndromes: May-Hegglin anomaly, Epstein syndrome, Fechtner syndrome, and Sebastian syndrome.² More than 30 MYH9 mutations have been identified, all of which encode for myosin-9, a subunit of myosin IIA,^1,3 that is a nonmuscle myosin needed for cell movement, shape, and cytokinesis. Although most cells use myosin IIA to IIC, certain cells, such as platelets and neutrophils, use myosin IIA exclusively.

In neutrophils of patients with MYH9-related disorder, nonfunctional myosin-9 clumps to form hallmark inclusion bodies, which are seen on the peripheral blood smear. Macrothrombocytopenia, another hallmark of MYH9-related disorder, also can be seen on the peripheral smear of all affected patients. Approximately 30%of patients develop clinical manifestations of the disorder (eg, bleeding, renal failure, hearing loss, presenile cataracts). Bleeding tendency usually is mild; epistaxis and menorrhagia are the most common hematologic manifestations.⁴

We attribute the lower leg hyperpigmentation in our patient to a severe phenotype of MYH9-related disorder. In addition to hyperpigmentation, our patient had menorrhagia requiring treatment with tranexamic acid, renal failure, and hearing loss, further pointing to a more severe phenotype. Furthermore, it is likely that our patient’s hyperpigmentation was made worse by hydroxychloroquine and a coexisting diagnosis of mixed connective tissue disease, which led to a propensity for increased vessel fragility in the setting of thrombocytopenia.

The workup of suspected MYH9-related disorder includes exclusion of iron-deficiency anemia, which can increase bleeding in patients with the disorder. The presence of small red blood cells (RBCs) in microcytic anemia and large platelets of MYH9-related disorder can lead to a situation in which platelets travel near the center of the lumen of blood vessels, while RBCs travel to the periphery. This decrease in the platelet-endothelium interaction increases the risk for bleeding. Our patient’s hemoglobin level was within reference range, without evidence of iron-deficiency anemia. Correction of iron-deficiency anemia, if applicable, can prevent bleeding brought on by the mechanism of decreased platelet-endothelium interaction and avoid unnecessary antiplatelet medication because of misdiagnosis based on an erroneous platelet count.

The workup of MYH9-related disorder also should include audiography, ophthalmologic examination, and renal function testing for hearing loss, cataracts, and renal disease, respectively. Referral to genetics also may be warranted.

It also is of clinical interest that automated cell counters may underestimate the count of abnormally large platelets in MYH9-related disorder, counting them as RBCs or white blood cells. The platelet count in MYH9-related disorder may be underestimated by 4-fold or greater.^4-7

Treatment of leg hyperpigmentation can prove challenging, given the location of dermal hemosiderin. Topical therapy likely is ineffective. Lasers and intense pulsed light therapy are treatment modalities to consider for the hyperpigmentation of MYH9-related disorder. There have been reports of improved cosmesis in dermal hemosiderin depositional disorders, such as venous stasis.⁴ Our patient was given ammonium lactate lotion to thicken collagen, possibly preventing future bleeding episodes.

To the Editor:

MYH9-related disorder is an autosomal-dominant disorder characterized by macrothrombocytopenia and neutrophil inclusions secondary to defective myosin-9.¹ We describe a case of lower leg hyperpigmentation secondary to hemosiderin deposition from MYH9-related disorder.

A 31-year-old woman with a history of MYH9-related disorder and mixed connective tissue disease presented to the outpatient dermatology clinic with asymptomatic brown patches on the lower legs (Figure) of 10 years’ duration. She also had epistaxis, hearing loss, renal disease, and menorrhagia secondary to MYH9-related disorder. The patient had been started on hydroxychloroquine 2 years earlier by rheumatology for mixed connective tissue disorder. A biopsy was not performed, given the risk of bleeding from thrombocytopenia. Ammonium lactate lotion was recommended for the leg patches. No further interventions were undertaken. At 6-month follow-up, hyperpigmentation on the lower legs was stable. The patient expressed no desire for cosmetic intervention.

Prior to discovery of a common gene, MYH9-related disorder was classified as 4 overlapping syndromes: May-Hegglin anomaly, Epstein syndrome, Fechtner syndrome, and Sebastian syndrome.² More than 30 MYH9 mutations have been identified, all of which encode for myosin-9, a subunit of myosin IIA,^1,3 that is a nonmuscle myosin needed for cell movement, shape, and cytokinesis. Although most cells use myosin IIA to IIC, certain cells, such as platelets and neutrophils, use myosin IIA exclusively.

In neutrophils of patients with MYH9-related disorder, nonfunctional myosin-9 clumps to form hallmark inclusion bodies, which are seen on the peripheral blood smear. Macrothrombocytopenia, another hallmark of MYH9-related disorder, also can be seen on the peripheral smear of all affected patients. Approximately 30%of patients develop clinical manifestations of the disorder (eg, bleeding, renal failure, hearing loss, presenile cataracts). Bleeding tendency usually is mild; epistaxis and menorrhagia are the most common hematologic manifestations.⁴

We attribute the lower leg hyperpigmentation in our patient to a severe phenotype of MYH9-related disorder. In addition to hyperpigmentation, our patient had menorrhagia requiring treatment with tranexamic acid, renal failure, and hearing loss, further pointing to a more severe phenotype. Furthermore, it is likely that our patient’s hyperpigmentation was made worse by hydroxychloroquine and a coexisting diagnosis of mixed connective tissue disease, which led to a propensity for increased vessel fragility in the setting of thrombocytopenia.

The workup of suspected MYH9-related disorder includes exclusion of iron-deficiency anemia, which can increase bleeding in patients with the disorder. The presence of small red blood cells (RBCs) in microcytic anemia and large platelets of MYH9-related disorder can lead to a situation in which platelets travel near the center of the lumen of blood vessels, while RBCs travel to the periphery. This decrease in the platelet-endothelium interaction increases the risk for bleeding. Our patient’s hemoglobin level was within reference range, without evidence of iron-deficiency anemia. Correction of iron-deficiency anemia, if applicable, can prevent bleeding brought on by the mechanism of decreased platelet-endothelium interaction and avoid unnecessary antiplatelet medication because of misdiagnosis based on an erroneous platelet count.

The workup of MYH9-related disorder also should include audiography, ophthalmologic examination, and renal function testing for hearing loss, cataracts, and renal disease, respectively. Referral to genetics also may be warranted.

It also is of clinical interest that automated cell counters may underestimate the count of abnormally large platelets in MYH9-related disorder, counting them as RBCs or white blood cells. The platelet count in MYH9-related disorder may be underestimated by 4-fold or greater.^4-7

Treatment of leg hyperpigmentation can prove challenging, given the location of dermal hemosiderin. Topical therapy likely is ineffective. Lasers and intense pulsed light therapy are treatment modalities to consider for the hyperpigmentation of MYH9-related disorder. There have been reports of improved cosmesis in dermal hemosiderin depositional disorders, such as venous stasis.⁴ Our patient was given ammonium lactate lotion to thicken collagen, possibly preventing future bleeding episodes.

To the Editor:

MYH9-related disorder is an autosomal-dominant disorder characterized by macrothrombocytopenia and neutrophil inclusions secondary to defective myosin-9.¹ We describe a case of lower leg hyperpigmentation secondary to hemosiderin deposition from MYH9-related disorder.

A 31-year-old woman with a history of MYH9-related disorder and mixed connective tissue disease presented to the outpatient dermatology clinic with asymptomatic brown patches on the lower legs (Figure) of 10 years’ duration. She also had epistaxis, hearing loss, renal disease, and menorrhagia secondary to MYH9-related disorder. The patient had been started on hydroxychloroquine 2 years earlier by rheumatology for mixed connective tissue disorder. A biopsy was not performed, given the risk of bleeding from thrombocytopenia. Ammonium lactate lotion was recommended for the leg patches. No further interventions were undertaken. At 6-month follow-up, hyperpigmentation on the lower legs was stable. The patient expressed no desire for cosmetic intervention.

Prior to discovery of a common gene, MYH9-related disorder was classified as 4 overlapping syndromes: May-Hegglin anomaly, Epstein syndrome, Fechtner syndrome, and Sebastian syndrome.² More than 30 MYH9 mutations have been identified, all of which encode for myosin-9, a subunit of myosin IIA,^1,3 that is a nonmuscle myosin needed for cell movement, shape, and cytokinesis. Although most cells use myosin IIA to IIC, certain cells, such as platelets and neutrophils, use myosin IIA exclusively.

In neutrophils of patients with MYH9-related disorder, nonfunctional myosin-9 clumps to form hallmark inclusion bodies, which are seen on the peripheral blood smear. Macrothrombocytopenia, another hallmark of MYH9-related disorder, also can be seen on the peripheral smear of all affected patients. Approximately 30%of patients develop clinical manifestations of the disorder (eg, bleeding, renal failure, hearing loss, presenile cataracts). Bleeding tendency usually is mild; epistaxis and menorrhagia are the most common hematologic manifestations.⁴

We attribute the lower leg hyperpigmentation in our patient to a severe phenotype of MYH9-related disorder. In addition to hyperpigmentation, our patient had menorrhagia requiring treatment with tranexamic acid, renal failure, and hearing loss, further pointing to a more severe phenotype. Furthermore, it is likely that our patient’s hyperpigmentation was made worse by hydroxychloroquine and a coexisting diagnosis of mixed connective tissue disease, which led to a propensity for increased vessel fragility in the setting of thrombocytopenia.

The workup of suspected MYH9-related disorder includes exclusion of iron-deficiency anemia, which can increase bleeding in patients with the disorder. The presence of small red blood cells (RBCs) in microcytic anemia and large platelets of MYH9-related disorder can lead to a situation in which platelets travel near the center of the lumen of blood vessels, while RBCs travel to the periphery. This decrease in the platelet-endothelium interaction increases the risk for bleeding. Our patient’s hemoglobin level was within reference range, without evidence of iron-deficiency anemia. Correction of iron-deficiency anemia, if applicable, can prevent bleeding brought on by the mechanism of decreased platelet-endothelium interaction and avoid unnecessary antiplatelet medication because of misdiagnosis based on an erroneous platelet count.

The workup of MYH9-related disorder also should include audiography, ophthalmologic examination, and renal function testing for hearing loss, cataracts, and renal disease, respectively. Referral to genetics also may be warranted.

It also is of clinical interest that automated cell counters may underestimate the count of abnormally large platelets in MYH9-related disorder, counting them as RBCs or white blood cells. The platelet count in MYH9-related disorder may be underestimated by 4-fold or greater.^4-7

Treatment of leg hyperpigmentation can prove challenging, given the location of dermal hemosiderin. Topical therapy likely is ineffective. Lasers and intense pulsed light therapy are treatment modalities to consider for the hyperpigmentation of MYH9-related disorder. There have been reports of improved cosmesis in dermal hemosiderin depositional disorders, such as venous stasis.⁴ Our patient was given ammonium lactate lotion to thicken collagen, possibly preventing future bleeding episodes.

The Diagnosis: Hailey-Hailey Disease (Benign Familial Chronic Pemphigus)

Our patient had a long-standing history of Hailey-Hailey disease, as confirmed by multiple prior skin biopsies at outside institutions as well as our affiliated site. He began treatment with oral doxycycline 50 mg twice daily for 2 weeks, triamcinolone cream 0.1% twice daily to the affected region, and aluminum acetate solution soaks and chlorhexidine wash daily along with petroleum jelly, which resulted in good control of the disease. The differential diagnosis of eroded plaques, particularly in the axillary, crural, and inframammary folds, is broad and includes candidiasis, inverse psoriasis, contact dermatitis, dermatophyte infection, pemphigus vegetans or foliaceus, and granular parakeratosis.

Hailey-Hailey disease is a genetic disorder with a prevalence of 1 in 50,000 individuals. Most patients develop symptoms during the second or third decades of life.1 Hailey-Hailey disease exhibits an autosomal-dominant pattern of inheritance secondary to mutation in the human ATP2C1 gene, which codes for the ATPase secretory pathway of the Ca²⁺ transporting pump type 1 (SPCA1) localized in the Golgi apparatus.² Altered SPCA1 protein reduces concentration of Ca²⁺ within the Golgi lumen, which in turn impairs the processing of junctional proteins needed for normal cell-to-cell adhesion.¹

Clinically, Hailey-Hailey disease is characterized by vesicular or erosive plaques that have a predilection for intertriginous areas of the body.¹ The primary lesions often are flaccid vesicles that easily rupture, leaving behind crusted erosions that spread peripherally. The lesions also can appear as macerated plaques resembling torn tissue paper, as in our case. Friction, heat, and sweat exacerbate the disease. Complications occur from secondary bacterial, fungal, and viral colonization. Malodor and vegetations can indicate bacterial or fungal infections and can lead to persistence of skin lesions. Herpes simplex virus infections can exacerbate preexisting lesions.³ Hailey-Hailey disease of the anogenital region also can be complicated by infection with oncogenic strains of human papillomavirus and lead to cutaneous squamous cell carcinoma.⁴

Hailey-Hailey disease histologically appears as suprabasal and intraepidermal keratinocyte acantholysis,⁵ which typically is widespread in the epidermis, with large areas of dyscohesion with a dilapidated brick wall-like appearance.¹ In more chronic lesions, epidermal hyperplasia, parakeratosis, and focal crusts may be observed. A moderate perivascular lymphocytic infiltrate can be observed in the superficial dermis. Direct immunofluorescence typically is negative.

Topical corticosteroids and antimicrobials are first-line therapies that often only provide temporary suppression. When the disease is refractory to topical therapies, intralesional corticosteroids may be attempted. There is no strong evidence to support the use of systemic therapy, aside from antimicrobial agents (eg, doxycycline) for the use of superinfections. In severe cases, immunomodulating therapies such as prednisone, cyclosporine, methotrexate, dapsone, alefacept, and oral retinoids may be effective.^6-8 Surgical therapy also can be considered for recalcitrant disease, including wide excision and grafting, though these techniques can be associated with morbidity.⁹

Superficial ablative techniques including dermabrasion, laser therapy with CO₂ and erbium-doped YAG, photodynamic therapy, and electron beam radiation have been shown to be effective modalities in severe cases.^5,9-11 It has been hypothesized that keratinocytes expressing the molecular defect are ablated, while the surrounding normal adnexal epithelium can regenerate normal epithelium. It also is thought that dermal fibrosis leads to better support of the diseased epidermis and decreases the risk for ulceration and fissuring.⁹

The Diagnosis: Hailey-Hailey Disease (Benign Familial Chronic Pemphigus)

Our patient had a long-standing history of Hailey-Hailey disease, as confirmed by multiple prior skin biopsies at outside institutions as well as our affiliated site. He began treatment with oral doxycycline 50 mg twice daily for 2 weeks, triamcinolone cream 0.1% twice daily to the affected region, and aluminum acetate solution soaks and chlorhexidine wash daily along with petroleum jelly, which resulted in good control of the disease. The differential diagnosis of eroded plaques, particularly in the axillary, crural, and inframammary folds, is broad and includes candidiasis, inverse psoriasis, contact dermatitis, dermatophyte infection, pemphigus vegetans or foliaceus, and granular parakeratosis.

Hailey-Hailey disease is a genetic disorder with a prevalence of 1 in 50,000 individuals. Most patients develop symptoms during the second or third decades of life.1 Hailey-Hailey disease exhibits an autosomal-dominant pattern of inheritance secondary to mutation in the human ATP2C1 gene, which codes for the ATPase secretory pathway of the Ca²⁺ transporting pump type 1 (SPCA1) localized in the Golgi apparatus.² Altered SPCA1 protein reduces concentration of Ca²⁺ within the Golgi lumen, which in turn impairs the processing of junctional proteins needed for normal cell-to-cell adhesion.¹

Clinically, Hailey-Hailey disease is characterized by vesicular or erosive plaques that have a predilection for intertriginous areas of the body.¹ The primary lesions often are flaccid vesicles that easily rupture, leaving behind crusted erosions that spread peripherally. The lesions also can appear as macerated plaques resembling torn tissue paper, as in our case. Friction, heat, and sweat exacerbate the disease. Complications occur from secondary bacterial, fungal, and viral colonization. Malodor and vegetations can indicate bacterial or fungal infections and can lead to persistence of skin lesions. Herpes simplex virus infections can exacerbate preexisting lesions.³ Hailey-Hailey disease of the anogenital region also can be complicated by infection with oncogenic strains of human papillomavirus and lead to cutaneous squamous cell carcinoma.⁴

Hailey-Hailey disease histologically appears as suprabasal and intraepidermal keratinocyte acantholysis,⁵ which typically is widespread in the epidermis, with large areas of dyscohesion with a dilapidated brick wall-like appearance.¹ In more chronic lesions, epidermal hyperplasia, parakeratosis, and focal crusts may be observed. A moderate perivascular lymphocytic infiltrate can be observed in the superficial dermis. Direct immunofluorescence typically is negative.

Topical corticosteroids and antimicrobials are first-line therapies that often only provide temporary suppression. When the disease is refractory to topical therapies, intralesional corticosteroids may be attempted. There is no strong evidence to support the use of systemic therapy, aside from antimicrobial agents (eg, doxycycline) for the use of superinfections. In severe cases, immunomodulating therapies such as prednisone, cyclosporine, methotrexate, dapsone, alefacept, and oral retinoids may be effective.^6-8 Surgical therapy also can be considered for recalcitrant disease, including wide excision and grafting, though these techniques can be associated with morbidity.⁹

Superficial ablative techniques including dermabrasion, laser therapy with CO₂ and erbium-doped YAG, photodynamic therapy, and electron beam radiation have been shown to be effective modalities in severe cases.^5,9-11 It has been hypothesized that keratinocytes expressing the molecular defect are ablated, while the surrounding normal adnexal epithelium can regenerate normal epithelium. It also is thought that dermal fibrosis leads to better support of the diseased epidermis and decreases the risk for ulceration and fissuring.⁹

The Diagnosis: Hailey-Hailey Disease (Benign Familial Chronic Pemphigus)

Our patient had a long-standing history of Hailey-Hailey disease, as confirmed by multiple prior skin biopsies at outside institutions as well as our affiliated site. He began treatment with oral doxycycline 50 mg twice daily for 2 weeks, triamcinolone cream 0.1% twice daily to the affected region, and aluminum acetate solution soaks and chlorhexidine wash daily along with petroleum jelly, which resulted in good control of the disease. The differential diagnosis of eroded plaques, particularly in the axillary, crural, and inframammary folds, is broad and includes candidiasis, inverse psoriasis, contact dermatitis, dermatophyte infection, pemphigus vegetans or foliaceus, and granular parakeratosis.

Hailey-Hailey disease is a genetic disorder with a prevalence of 1 in 50,000 individuals. Most patients develop symptoms during the second or third decades of life.1 Hailey-Hailey disease exhibits an autosomal-dominant pattern of inheritance secondary to mutation in the human ATP2C1 gene, which codes for the ATPase secretory pathway of the Ca²⁺ transporting pump type 1 (SPCA1) localized in the Golgi apparatus.² Altered SPCA1 protein reduces concentration of Ca²⁺ within the Golgi lumen, which in turn impairs the processing of junctional proteins needed for normal cell-to-cell adhesion.¹

Clinically, Hailey-Hailey disease is characterized by vesicular or erosive plaques that have a predilection for intertriginous areas of the body.¹ The primary lesions often are flaccid vesicles that easily rupture, leaving behind crusted erosions that spread peripherally. The lesions also can appear as macerated plaques resembling torn tissue paper, as in our case. Friction, heat, and sweat exacerbate the disease. Complications occur from secondary bacterial, fungal, and viral colonization. Malodor and vegetations can indicate bacterial or fungal infections and can lead to persistence of skin lesions. Herpes simplex virus infections can exacerbate preexisting lesions.³ Hailey-Hailey disease of the anogenital region also can be complicated by infection with oncogenic strains of human papillomavirus and lead to cutaneous squamous cell carcinoma.⁴

Hailey-Hailey disease histologically appears as suprabasal and intraepidermal keratinocyte acantholysis,⁵ which typically is widespread in the epidermis, with large areas of dyscohesion with a dilapidated brick wall-like appearance.¹ In more chronic lesions, epidermal hyperplasia, parakeratosis, and focal crusts may be observed. A moderate perivascular lymphocytic infiltrate can be observed in the superficial dermis. Direct immunofluorescence typically is negative.

Topical corticosteroids and antimicrobials are first-line therapies that often only provide temporary suppression. When the disease is refractory to topical therapies, intralesional corticosteroids may be attempted. There is no strong evidence to support the use of systemic therapy, aside from antimicrobial agents (eg, doxycycline) for the use of superinfections. In severe cases, immunomodulating therapies such as prednisone, cyclosporine, methotrexate, dapsone, alefacept, and oral retinoids may be effective.^6-8 Surgical therapy also can be considered for recalcitrant disease, including wide excision and grafting, though these techniques can be associated with morbidity.⁹

Superficial ablative techniques including dermabrasion, laser therapy with CO₂ and erbium-doped YAG, photodynamic therapy, and electron beam radiation have been shown to be effective modalities in severe cases.^5,9-11 It has been hypothesized that keratinocytes expressing the molecular defect are ablated, while the surrounding normal adnexal epithelium can regenerate normal epithelium. It also is thought that dermal fibrosis leads to better support of the diseased epidermis and decreases the risk for ulceration and fissuring.⁹

The Diagnosis: Corticosteroid-Induced Hypopigmentation

This patient received several intralesional injections of triamcinolone acetonide once monthly for treatment of the keloid scar on the left ear at an outside institution. There was improvement in the size of the keloid over time. On physical examination during the most recent visit there was a prominent streak of hypopigmentation and atrophy near the corticosteroid injection site with extension to the postauricular region. There also was telangiectasia noted within the area of hypopigmentation. Intralesional triamcinolone injections were discontinued and the patient was advised to return for monitoring.

Intra-articular and intralesional corticosteroid injections frequently are used by clinicians. Cutaneous complications associated with these injections include atrophy, pigmentary changes, hypersensitivity reactions, flushing, cellulitis, and necrotizing fasciitis. Tendon rupture also has been reported.¹

There are several case reports in the literature describing hypopigmentation and/or subcutaneous atrophy after intralesional or intra-articular corticosteroid injections. A variety of underlying conditions were treated including alopecia areata, keloids, rheumatoid arthritis, de Quervain tendonitis, and psoriasis.^2-6 The lesions typically are described as linear rays of atrophy and hypopigmentation at or near the injection site, with some cases noting extension along lymph channels and proximal veins.^4,6 There usually is no associated pruritus or pain.³ This phenomenon can be seen after single or multiple injections.^4,6

Extension of hypopigmentation from the site of injection has been postulated to be due to venous or lymphatic uptake.^2,4-6 The mechanism of hypopigmentation is not known. Biopsy of a previously described case showed intact melanocytes along the dermoepidermal junction.² Biopsy from another case revealed a decrease in melanin staining, which suggests a decrease in number or activity of melanocytes.⁴ It was proposed that hypopigmentation was secondary to loss of melanocyte function instead of loss of melanocytes.² Spontaneous improvement or resolution of the hypopigmentation were noted in some cases ranging from 1 month to 1 year after initial presentation, but the hypopigmentation also can be persistent.^3-6

Hypopigmented sarcoidosis and hypopigmented mycosis fungoides, both often present on dark-skinned individuals, are included in the differential diagnosis. Hypopigmented sarcoidosis presents with hypopigmented macules or patches, some with central papules, and hypopigmented mycosis fungoides presents with hypopigmented patches or plaques with fine scale and onset often in childhood or adolescence.^7,8 Morphea can present with an initial inflammatory stage that develops into a sclerotic firm plaque or nodule with hyperpigmentation or hypopigmentation.⁹ Vitiligo usually presents with depigmented macules or patches and depigmented hair within the lesion.¹⁰

The Diagnosis: Corticosteroid-Induced Hypopigmentation

This patient received several intralesional injections of triamcinolone acetonide once monthly for treatment of the keloid scar on the left ear at an outside institution. There was improvement in the size of the keloid over time. On physical examination during the most recent visit there was a prominent streak of hypopigmentation and atrophy near the corticosteroid injection site with extension to the postauricular region. There also was telangiectasia noted within the area of hypopigmentation. Intralesional triamcinolone injections were discontinued and the patient was advised to return for monitoring.

Intra-articular and intralesional corticosteroid injections frequently are used by clinicians. Cutaneous complications associated with these injections include atrophy, pigmentary changes, hypersensitivity reactions, flushing, cellulitis, and necrotizing fasciitis. Tendon rupture also has been reported.¹

There are several case reports in the literature describing hypopigmentation and/or subcutaneous atrophy after intralesional or intra-articular corticosteroid injections. A variety of underlying conditions were treated including alopecia areata, keloids, rheumatoid arthritis, de Quervain tendonitis, and psoriasis.^2-6 The lesions typically are described as linear rays of atrophy and hypopigmentation at or near the injection site, with some cases noting extension along lymph channels and proximal veins.^4,6 There usually is no associated pruritus or pain.³ This phenomenon can be seen after single or multiple injections.^4,6

Extension of hypopigmentation from the site of injection has been postulated to be due to venous or lymphatic uptake.^2,4-6 The mechanism of hypopigmentation is not known. Biopsy of a previously described case showed intact melanocytes along the dermoepidermal junction.² Biopsy from another case revealed a decrease in melanin staining, which suggests a decrease in number or activity of melanocytes.⁴ It was proposed that hypopigmentation was secondary to loss of melanocyte function instead of loss of melanocytes.² Spontaneous improvement or resolution of the hypopigmentation were noted in some cases ranging from 1 month to 1 year after initial presentation, but the hypopigmentation also can be persistent.^3-6

Hypopigmented sarcoidosis and hypopigmented mycosis fungoides, both often present on dark-skinned individuals, are included in the differential diagnosis. Hypopigmented sarcoidosis presents with hypopigmented macules or patches, some with central papules, and hypopigmented mycosis fungoides presents with hypopigmented patches or plaques with fine scale and onset often in childhood or adolescence.^7,8 Morphea can present with an initial inflammatory stage that develops into a sclerotic firm plaque or nodule with hyperpigmentation or hypopigmentation.⁹ Vitiligo usually presents with depigmented macules or patches and depigmented hair within the lesion.¹⁰

The Diagnosis: Corticosteroid-Induced Hypopigmentation

This patient received several intralesional injections of triamcinolone acetonide once monthly for treatment of the keloid scar on the left ear at an outside institution. There was improvement in the size of the keloid over time. On physical examination during the most recent visit there was a prominent streak of hypopigmentation and atrophy near the corticosteroid injection site with extension to the postauricular region. There also was telangiectasia noted within the area of hypopigmentation. Intralesional triamcinolone injections were discontinued and the patient was advised to return for monitoring.

Intra-articular and intralesional corticosteroid injections frequently are used by clinicians. Cutaneous complications associated with these injections include atrophy, pigmentary changes, hypersensitivity reactions, flushing, cellulitis, and necrotizing fasciitis. Tendon rupture also has been reported.¹

There are several case reports in the literature describing hypopigmentation and/or subcutaneous atrophy after intralesional or intra-articular corticosteroid injections. A variety of underlying conditions were treated including alopecia areata, keloids, rheumatoid arthritis, de Quervain tendonitis, and psoriasis.^2-6 The lesions typically are described as linear rays of atrophy and hypopigmentation at or near the injection site, with some cases noting extension along lymph channels and proximal veins.^4,6 There usually is no associated pruritus or pain.³ This phenomenon can be seen after single or multiple injections.^4,6

Extension of hypopigmentation from the site of injection has been postulated to be due to venous or lymphatic uptake.^2,4-6 The mechanism of hypopigmentation is not known. Biopsy of a previously described case showed intact melanocytes along the dermoepidermal junction.² Biopsy from another case revealed a decrease in melanin staining, which suggests a decrease in number or activity of melanocytes.⁴ It was proposed that hypopigmentation was secondary to loss of melanocyte function instead of loss of melanocytes.² Spontaneous improvement or resolution of the hypopigmentation were noted in some cases ranging from 1 month to 1 year after initial presentation, but the hypopigmentation also can be persistent.^3-6

Hypopigmented sarcoidosis and hypopigmented mycosis fungoides, both often present on dark-skinned individuals, are included in the differential diagnosis. Hypopigmented sarcoidosis presents with hypopigmented macules or patches, some with central papules, and hypopigmented mycosis fungoides presents with hypopigmented patches or plaques with fine scale and onset often in childhood or adolescence.^7,8 Morphea can present with an initial inflammatory stage that develops into a sclerotic firm plaque or nodule with hyperpigmentation or hypopigmentation.⁹ Vitiligo usually presents with depigmented macules or patches and depigmented hair within the lesion.¹⁰