Pediatric Dermatology Consult - August 2016

Dr. Catalina Matiz and David Ginsberg describe the diagnosis and treatment of discoid lupus erythematosus in children.

Discoid lupus erythematosus

Discoid lupus erythematosus (DLE) is a relatively common form of chronic cutaneous lupus, although its presentation in children is rare. The typical presentation of DLE is well-circumscribed, indurated, sometimes scaly round or oval plaques with pigmentary change, often red to purple in color. DLE also is clinically associated with telangiectasia, scarring, and follicular plugging, which has a characteristic appearance of “carpet tacking” beneath the scale.

When left untreated, these lesions may result in areas of long-term hypo- or hyperpigmentation, as well as atrophy and scarring.1 These cutaneous manifestations often are exacerbated by UV light exposure. This is particularly problematic because DLE most often affects the face, although lesions also can be found on the scalp, ears, trunk, extremities, and in the mouth as well.

Currently, there are few studies looking specifically at DLE in children and, based on the studies that have been done, there appear to be several important differences between the adult and pediatric populations. DLE affects women more than twice as much as men in the adult population, but reports vary as to whether this female predominance carries over to affected children.^2,3 Adults with DLE rarely have a family history of systemic lupus erythematosus (SLE), with rates reported between 1% and 4.4%. In children, however, the reported rates of family history increase tenfold to 11%-40%.²

One study showed that children with DLE progress to SLE at a rate of 23.5%-26%, which is higher than reported rates in adults of 5%-20%.^2,4 For this reason, repeated laboratory studies are essential in the follow-up of children diagnosed with DLE, given the possibility of a transition to systemic disease, particularly within the first year of diagnosis. Disseminated lesions can be a red flag for future progression to SLE.²

Differential diagnosis

The differential diagnosis of infiltrated annular lesions on the face and ears should include conditions such as tinea faciae, seborrheic dermatitis, granuloma annulare, cutaneous lymphoma, sarcoidosis, and leishmaniasis. When the lesions are present on the ear or nose, as in the case of this patient, relapsing polychonritis also may be considered.

Although histology plays a large part in diagnosing DLE, clinical presentation and recognizing the need for a biopsy are important.⁵ Tinea faciei, a fungal infection of the face, can present as erythematous scaly plaques.⁶ A thorough history and physical exam are important in differentiating tinea faciei from DLE because the former often begins as a small scaly papule that annularly expands outward to form larger plaque with scale around the outer rim, as opposed to DLE, which may have an adherent scale across the entire plaque.⁵ A potassium hydroxide (KOH) preparation of scraped scale from one of the lesions or a fungal culture can confirm the diagnosis of tinea faciei.⁶

Seborrheic dermatitis can be localized to the face and scalp and presents with greasy yellow scales. Early lesions of DLE can be difficult to differentiate from seborrheic dermatitis.

Infiltrated annular lesions on the face may represent granulomatous conditions such as granuloma annulare or sarcoidosis, but these lesions usually lack the presence of scale that can be seen in DLE.

Relapsing polychondritis presents as intermittent episodes of cartilage inflammation, usually affecting the cartilage of the ear, nose, and respiratory tract. Areas affected do not show changes on the surface of the skin as it occurs in DLE lesions.

As mentioned above, family history of SLE could indicate a potential for DLE in a small percentage of patients, but the clinical feature of the scaly plaques with the carpet tacking underneath the scale, caused by follicular plugging, is helpful in making the diagnosis clinically. Ultimately, the best way to differentiate anything resembling DLE is through histology and direct immunofluorescence (DIF). Histologic findings in DLE include epidermal atrophy, basal membrane cell vacuolization, hyperkeratosis, parakeratosis, corneal plugs, pseudoscysts, and acanthosis.³ The lupus band test is done using DIF and is a widely used tool for making the diagnosis of DLE based on the distribution of immunoglobulin deposition in the basement membrane zone.⁷

Treatment

Without timely diagnosis and treatment of DLE, the lesions can progress to scarring and atrophy, leading to a decreased quality of life. UV light exposure and smoking can exacerbate DLE, so sun protection and smoking cessation are both recommended in patients with DLE, although, admittedly, the latter is less relevant in the pediatric population.¹ Topical, intralesional, or systemic corticosteroids, with or without antimalarials, are the first line therapy for the management of DLE.¹ For refractory cases, some reports document the use of topical calcineurin inhibitors, dapsone, methotrexate, and topical or systemic retinoids.¹ For severe cases, intravenous immunoglobulin, ustekinumab, and rituximab also may be used.¹

References

1. Dermatol Ther. 2016 Apr 12 Epub.
2. Pediatr Dermatol. 2008 Mar-Apr;25(2):163-7.
3. Pediatr Dermatol. 2003 Mar-Apr;20(2):103-7.
4. J Am Acad Dermatol. 2015 Apr;72(4):628-33.
5. Pediatr Dermatol. 2016 Mar-Apr;33(2):200-8.
6. Pediatr Clin North Am. 2014 Apr;61(2):443-55.
7. Am J Dermatopathol. 2016 Feb;38(2):121-3.

Dr. Matiz is assistant professor of dermatology at Rady Children’s Hospital San Diego–University of California, San Diego, and Mr. Ginsberg is a research associate at the hospital. Dr. Matiz and Mr. Ginsberg said they have no relevant financial disclosures.

Dr. Catalina Matiz and David Ginsberg describe the diagnosis and treatment of discoid lupus erythematosus in children.

Discoid lupus erythematosus

Discoid lupus erythematosus (DLE) is a relatively common form of chronic cutaneous lupus, although its presentation in children is rare. The typical presentation of DLE is well-circumscribed, indurated, sometimes scaly round or oval plaques with pigmentary change, often red to purple in color. DLE also is clinically associated with telangiectasia, scarring, and follicular plugging, which has a characteristic appearance of “carpet tacking” beneath the scale.

When left untreated, these lesions may result in areas of long-term hypo- or hyperpigmentation, as well as atrophy and scarring.1 These cutaneous manifestations often are exacerbated by UV light exposure. This is particularly problematic because DLE most often affects the face, although lesions also can be found on the scalp, ears, trunk, extremities, and in the mouth as well.

Currently, there are few studies looking specifically at DLE in children and, based on the studies that have been done, there appear to be several important differences between the adult and pediatric populations. DLE affects women more than twice as much as men in the adult population, but reports vary as to whether this female predominance carries over to affected children.^2,3 Adults with DLE rarely have a family history of systemic lupus erythematosus (SLE), with rates reported between 1% and 4.4%. In children, however, the reported rates of family history increase tenfold to 11%-40%.²

One study showed that children with DLE progress to SLE at a rate of 23.5%-26%, which is higher than reported rates in adults of 5%-20%.^2,4 For this reason, repeated laboratory studies are essential in the follow-up of children diagnosed with DLE, given the possibility of a transition to systemic disease, particularly within the first year of diagnosis. Disseminated lesions can be a red flag for future progression to SLE.²

Differential diagnosis

The differential diagnosis of infiltrated annular lesions on the face and ears should include conditions such as tinea faciae, seborrheic dermatitis, granuloma annulare, cutaneous lymphoma, sarcoidosis, and leishmaniasis. When the lesions are present on the ear or nose, as in the case of this patient, relapsing polychonritis also may be considered.

Although histology plays a large part in diagnosing DLE, clinical presentation and recognizing the need for a biopsy are important.⁵ Tinea faciei, a fungal infection of the face, can present as erythematous scaly plaques.⁶ A thorough history and physical exam are important in differentiating tinea faciei from DLE because the former often begins as a small scaly papule that annularly expands outward to form larger plaque with scale around the outer rim, as opposed to DLE, which may have an adherent scale across the entire plaque.⁵ A potassium hydroxide (KOH) preparation of scraped scale from one of the lesions or a fungal culture can confirm the diagnosis of tinea faciei.⁶

Seborrheic dermatitis can be localized to the face and scalp and presents with greasy yellow scales. Early lesions of DLE can be difficult to differentiate from seborrheic dermatitis.

Infiltrated annular lesions on the face may represent granulomatous conditions such as granuloma annulare or sarcoidosis, but these lesions usually lack the presence of scale that can be seen in DLE.

Relapsing polychondritis presents as intermittent episodes of cartilage inflammation, usually affecting the cartilage of the ear, nose, and respiratory tract. Areas affected do not show changes on the surface of the skin as it occurs in DLE lesions.

As mentioned above, family history of SLE could indicate a potential for DLE in a small percentage of patients, but the clinical feature of the scaly plaques with the carpet tacking underneath the scale, caused by follicular plugging, is helpful in making the diagnosis clinically. Ultimately, the best way to differentiate anything resembling DLE is through histology and direct immunofluorescence (DIF). Histologic findings in DLE include epidermal atrophy, basal membrane cell vacuolization, hyperkeratosis, parakeratosis, corneal plugs, pseudoscysts, and acanthosis.³ The lupus band test is done using DIF and is a widely used tool for making the diagnosis of DLE based on the distribution of immunoglobulin deposition in the basement membrane zone.⁷

Treatment

Without timely diagnosis and treatment of DLE, the lesions can progress to scarring and atrophy, leading to a decreased quality of life. UV light exposure and smoking can exacerbate DLE, so sun protection and smoking cessation are both recommended in patients with DLE, although, admittedly, the latter is less relevant in the pediatric population.¹ Topical, intralesional, or systemic corticosteroids, with or without antimalarials, are the first line therapy for the management of DLE.¹ For refractory cases, some reports document the use of topical calcineurin inhibitors, dapsone, methotrexate, and topical or systemic retinoids.¹ For severe cases, intravenous immunoglobulin, ustekinumab, and rituximab also may be used.¹

References

1. Dermatol Ther. 2016 Apr 12 Epub.
2. Pediatr Dermatol. 2008 Mar-Apr;25(2):163-7.
3. Pediatr Dermatol. 2003 Mar-Apr;20(2):103-7.
4. J Am Acad Dermatol. 2015 Apr;72(4):628-33.
5. Pediatr Dermatol. 2016 Mar-Apr;33(2):200-8.
6. Pediatr Clin North Am. 2014 Apr;61(2):443-55.
7. Am J Dermatopathol. 2016 Feb;38(2):121-3.

Dr. Matiz is assistant professor of dermatology at Rady Children’s Hospital San Diego–University of California, San Diego, and Mr. Ginsberg is a research associate at the hospital. Dr. Matiz and Mr. Ginsberg said they have no relevant financial disclosures.

Dr. Catalina Matiz and David Ginsberg describe the diagnosis and treatment of discoid lupus erythematosus in children.

Discoid lupus erythematosus

Discoid lupus erythematosus (DLE) is a relatively common form of chronic cutaneous lupus, although its presentation in children is rare. The typical presentation of DLE is well-circumscribed, indurated, sometimes scaly round or oval plaques with pigmentary change, often red to purple in color. DLE also is clinically associated with telangiectasia, scarring, and follicular plugging, which has a characteristic appearance of “carpet tacking” beneath the scale.

When left untreated, these lesions may result in areas of long-term hypo- or hyperpigmentation, as well as atrophy and scarring.1 These cutaneous manifestations often are exacerbated by UV light exposure. This is particularly problematic because DLE most often affects the face, although lesions also can be found on the scalp, ears, trunk, extremities, and in the mouth as well.

Currently, there are few studies looking specifically at DLE in children and, based on the studies that have been done, there appear to be several important differences between the adult and pediatric populations. DLE affects women more than twice as much as men in the adult population, but reports vary as to whether this female predominance carries over to affected children.^2,3 Adults with DLE rarely have a family history of systemic lupus erythematosus (SLE), with rates reported between 1% and 4.4%. In children, however, the reported rates of family history increase tenfold to 11%-40%.²

One study showed that children with DLE progress to SLE at a rate of 23.5%-26%, which is higher than reported rates in adults of 5%-20%.^2,4 For this reason, repeated laboratory studies are essential in the follow-up of children diagnosed with DLE, given the possibility of a transition to systemic disease, particularly within the first year of diagnosis. Disseminated lesions can be a red flag for future progression to SLE.²

Differential diagnosis

The differential diagnosis of infiltrated annular lesions on the face and ears should include conditions such as tinea faciae, seborrheic dermatitis, granuloma annulare, cutaneous lymphoma, sarcoidosis, and leishmaniasis. When the lesions are present on the ear or nose, as in the case of this patient, relapsing polychonritis also may be considered.

Although histology plays a large part in diagnosing DLE, clinical presentation and recognizing the need for a biopsy are important.⁵ Tinea faciei, a fungal infection of the face, can present as erythematous scaly plaques.⁶ A thorough history and physical exam are important in differentiating tinea faciei from DLE because the former often begins as a small scaly papule that annularly expands outward to form larger plaque with scale around the outer rim, as opposed to DLE, which may have an adherent scale across the entire plaque.⁵ A potassium hydroxide (KOH) preparation of scraped scale from one of the lesions or a fungal culture can confirm the diagnosis of tinea faciei.⁶

Seborrheic dermatitis can be localized to the face and scalp and presents with greasy yellow scales. Early lesions of DLE can be difficult to differentiate from seborrheic dermatitis.

Infiltrated annular lesions on the face may represent granulomatous conditions such as granuloma annulare or sarcoidosis, but these lesions usually lack the presence of scale that can be seen in DLE.

Relapsing polychondritis presents as intermittent episodes of cartilage inflammation, usually affecting the cartilage of the ear, nose, and respiratory tract. Areas affected do not show changes on the surface of the skin as it occurs in DLE lesions.

As mentioned above, family history of SLE could indicate a potential for DLE in a small percentage of patients, but the clinical feature of the scaly plaques with the carpet tacking underneath the scale, caused by follicular plugging, is helpful in making the diagnosis clinically. Ultimately, the best way to differentiate anything resembling DLE is through histology and direct immunofluorescence (DIF). Histologic findings in DLE include epidermal atrophy, basal membrane cell vacuolization, hyperkeratosis, parakeratosis, corneal plugs, pseudoscysts, and acanthosis.³ The lupus band test is done using DIF and is a widely used tool for making the diagnosis of DLE based on the distribution of immunoglobulin deposition in the basement membrane zone.⁷

Treatment

Without timely diagnosis and treatment of DLE, the lesions can progress to scarring and atrophy, leading to a decreased quality of life. UV light exposure and smoking can exacerbate DLE, so sun protection and smoking cessation are both recommended in patients with DLE, although, admittedly, the latter is less relevant in the pediatric population.¹ Topical, intralesional, or systemic corticosteroids, with or without antimalarials, are the first line therapy for the management of DLE.¹ For refractory cases, some reports document the use of topical calcineurin inhibitors, dapsone, methotrexate, and topical or systemic retinoids.¹ For severe cases, intravenous immunoglobulin, ustekinumab, and rituximab also may be used.¹

References

1. Dermatol Ther. 2016 Apr 12 Epub.
2. Pediatr Dermatol. 2008 Mar-Apr;25(2):163-7.
3. Pediatr Dermatol. 2003 Mar-Apr;20(2):103-7.
4. J Am Acad Dermatol. 2015 Apr;72(4):628-33.
5. Pediatr Dermatol. 2016 Mar-Apr;33(2):200-8.
6. Pediatr Clin North Am. 2014 Apr;61(2):443-55.
7. Am J Dermatopathol. 2016 Feb;38(2):121-3.

Dr. Matiz is assistant professor of dermatology at Rady Children’s Hospital San Diego–University of California, San Diego, and Mr. Ginsberg is a research associate at the hospital. Dr. Matiz and Mr. Ginsberg said they have no relevant financial disclosures.