Article Type
Changed
Fri, 04/12/2024 - 10:35

KOH analysis of the scales from the scalp areas revealed no fungal elements. Given the observed erythema and scaling, a punch biopsy was conducted. Histopathological examination of the biopsy sample displayed interface inflammation affecting both the infundibular and lower portions of hair follicles. The presence of folliculosebaceous units transitioning from intermediate to terminal size follicles was noted. A perifollicular, peri eccrine, superficial, and deep perivascular lymphoplasmacytic infiltrate was identified, alongside increased dermal mucin, findings consistent with a diagnosis of discoid lupus erythematosus.

Subsequent laboratory investigations were largely unremarkable, except for an elevated ANA titer (1:320, with a speckled pattern). The patient was initiated on a treatment regimen comprising intralesional triamcinolone and oral hydroxychloroquine (Plaquenil).

Patch of alopecia with some associated erythema and scale
Dr. Matiz

 

Discussion

Pediatric discoid lupus erythematosus (DLE) is a rare but chronic autoimmune condition, representing one of the most prevalent forms of cutaneous lupus erythematosus. It predominantly affects adults, yet pediatric cases account for 5%-7% of DLE diagnoses, with a significant predominance in females. Pediatric scalp DLE is particularly concerning due to its potential for causing scarring and permanent hair loss, which can significantly impact the psychological wellbeing of affected children.

The pathogenesis of DLE is multifactorial, involving genetic predispositions, environmental factors like UV light exposure, and immunological mechanisms leading to skin damage.

In children, DLE typically presents as well-demarcated, erythematous plaques with scale and follicular plugging, primarily affecting the scalp. Lesions may also exhibit changes in pigmentation, atrophy, and telangiectasia. The scalp involvement often leads to scarring alopecia, which can be distressing for pediatric patients. Unlike systemic lupus erythematosus (SLE), DLE is usually limited to the skin without systemic involvement. The progression of DLE to systemic lupus erythematosus in children has been previously described to be 22.2%. In a recent report of 201 pediatric cases of DLE, 12% of the cases progressed to systemic lupus erythematosus (SLE) and 14.5% had concurrent SLE. The onset of symptoms before the age of 10 years was the only statistically significant predictor for progression to SLE. Pruritus is a common symptom and may be correlated with disease activity.

The differential diagnosis for this patient encompassed a variety of conditions, including tinea capitis, alopecia areata, trichotillomania, and lichen planopilaris, each considered based on clinical presentation but ultimately excluded through clinical, microscopic, and biopsy findings.

Dr. Catalina Matiz, a pediatric dermatologist at Southern California Permanente Medical Group, San Diego
Dr. Catalina Matiz


Management strategies for pediatric scalp DLE aim at preventing disease progression, minimizing scarring, and addressing aesthetic concerns. These include the use of topical and intralesional corticosteroids, calcineurin inhibitors, and antimalarial agents like hydroxychloroquine, alongside stringent photoprotection to mitigate UV-triggered exacerbations.
 

Conclusion

The prognosis for pediatric scalp DLE can be favorable with timely and appropriate management, underscoring the importance of early diagnosis and intervention to prevent scarring and hair loss. However, ongoing surveillance is crucial for monitoring potential progression to systemic lupus erythematosus, albeit a low-risk transformation.

Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego.

References

1. George PM and Tunnessen WW. Childhood discoid lupus erythematosus. Arch Dermatol. 1993;129(5):613-617.

2. Hawat T et al. Pediatric discoid lupus erythematosus: Short report. Dermatol Ther. 2022 Jan;35(1):e15170. doi: 10.1111/dth.15170.

3. Arkin LM et al. Practice-based differences in paediatric discoid lupus erythematosus. Br J Dermatol. 2019 Oct;181(4):805-810. doi: 10.1111/bjd.17780.

Publications
Topics
Sections

KOH analysis of the scales from the scalp areas revealed no fungal elements. Given the observed erythema and scaling, a punch biopsy was conducted. Histopathological examination of the biopsy sample displayed interface inflammation affecting both the infundibular and lower portions of hair follicles. The presence of folliculosebaceous units transitioning from intermediate to terminal size follicles was noted. A perifollicular, peri eccrine, superficial, and deep perivascular lymphoplasmacytic infiltrate was identified, alongside increased dermal mucin, findings consistent with a diagnosis of discoid lupus erythematosus.

Subsequent laboratory investigations were largely unremarkable, except for an elevated ANA titer (1:320, with a speckled pattern). The patient was initiated on a treatment regimen comprising intralesional triamcinolone and oral hydroxychloroquine (Plaquenil).

Patch of alopecia with some associated erythema and scale
Dr. Matiz

 

Discussion

Pediatric discoid lupus erythematosus (DLE) is a rare but chronic autoimmune condition, representing one of the most prevalent forms of cutaneous lupus erythematosus. It predominantly affects adults, yet pediatric cases account for 5%-7% of DLE diagnoses, with a significant predominance in females. Pediatric scalp DLE is particularly concerning due to its potential for causing scarring and permanent hair loss, which can significantly impact the psychological wellbeing of affected children.

The pathogenesis of DLE is multifactorial, involving genetic predispositions, environmental factors like UV light exposure, and immunological mechanisms leading to skin damage.

In children, DLE typically presents as well-demarcated, erythematous plaques with scale and follicular plugging, primarily affecting the scalp. Lesions may also exhibit changes in pigmentation, atrophy, and telangiectasia. The scalp involvement often leads to scarring alopecia, which can be distressing for pediatric patients. Unlike systemic lupus erythematosus (SLE), DLE is usually limited to the skin without systemic involvement. The progression of DLE to systemic lupus erythematosus in children has been previously described to be 22.2%. In a recent report of 201 pediatric cases of DLE, 12% of the cases progressed to systemic lupus erythematosus (SLE) and 14.5% had concurrent SLE. The onset of symptoms before the age of 10 years was the only statistically significant predictor for progression to SLE. Pruritus is a common symptom and may be correlated with disease activity.

The differential diagnosis for this patient encompassed a variety of conditions, including tinea capitis, alopecia areata, trichotillomania, and lichen planopilaris, each considered based on clinical presentation but ultimately excluded through clinical, microscopic, and biopsy findings.

Dr. Catalina Matiz, a pediatric dermatologist at Southern California Permanente Medical Group, San Diego
Dr. Catalina Matiz


Management strategies for pediatric scalp DLE aim at preventing disease progression, minimizing scarring, and addressing aesthetic concerns. These include the use of topical and intralesional corticosteroids, calcineurin inhibitors, and antimalarial agents like hydroxychloroquine, alongside stringent photoprotection to mitigate UV-triggered exacerbations.
 

Conclusion

The prognosis for pediatric scalp DLE can be favorable with timely and appropriate management, underscoring the importance of early diagnosis and intervention to prevent scarring and hair loss. However, ongoing surveillance is crucial for monitoring potential progression to systemic lupus erythematosus, albeit a low-risk transformation.

Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego.

References

1. George PM and Tunnessen WW. Childhood discoid lupus erythematosus. Arch Dermatol. 1993;129(5):613-617.

2. Hawat T et al. Pediatric discoid lupus erythematosus: Short report. Dermatol Ther. 2022 Jan;35(1):e15170. doi: 10.1111/dth.15170.

3. Arkin LM et al. Practice-based differences in paediatric discoid lupus erythematosus. Br J Dermatol. 2019 Oct;181(4):805-810. doi: 10.1111/bjd.17780.

KOH analysis of the scales from the scalp areas revealed no fungal elements. Given the observed erythema and scaling, a punch biopsy was conducted. Histopathological examination of the biopsy sample displayed interface inflammation affecting both the infundibular and lower portions of hair follicles. The presence of folliculosebaceous units transitioning from intermediate to terminal size follicles was noted. A perifollicular, peri eccrine, superficial, and deep perivascular lymphoplasmacytic infiltrate was identified, alongside increased dermal mucin, findings consistent with a diagnosis of discoid lupus erythematosus.

Subsequent laboratory investigations were largely unremarkable, except for an elevated ANA titer (1:320, with a speckled pattern). The patient was initiated on a treatment regimen comprising intralesional triamcinolone and oral hydroxychloroquine (Plaquenil).

Patch of alopecia with some associated erythema and scale
Dr. Matiz

 

Discussion

Pediatric discoid lupus erythematosus (DLE) is a rare but chronic autoimmune condition, representing one of the most prevalent forms of cutaneous lupus erythematosus. It predominantly affects adults, yet pediatric cases account for 5%-7% of DLE diagnoses, with a significant predominance in females. Pediatric scalp DLE is particularly concerning due to its potential for causing scarring and permanent hair loss, which can significantly impact the psychological wellbeing of affected children.

The pathogenesis of DLE is multifactorial, involving genetic predispositions, environmental factors like UV light exposure, and immunological mechanisms leading to skin damage.

In children, DLE typically presents as well-demarcated, erythematous plaques with scale and follicular plugging, primarily affecting the scalp. Lesions may also exhibit changes in pigmentation, atrophy, and telangiectasia. The scalp involvement often leads to scarring alopecia, which can be distressing for pediatric patients. Unlike systemic lupus erythematosus (SLE), DLE is usually limited to the skin without systemic involvement. The progression of DLE to systemic lupus erythematosus in children has been previously described to be 22.2%. In a recent report of 201 pediatric cases of DLE, 12% of the cases progressed to systemic lupus erythematosus (SLE) and 14.5% had concurrent SLE. The onset of symptoms before the age of 10 years was the only statistically significant predictor for progression to SLE. Pruritus is a common symptom and may be correlated with disease activity.

The differential diagnosis for this patient encompassed a variety of conditions, including tinea capitis, alopecia areata, trichotillomania, and lichen planopilaris, each considered based on clinical presentation but ultimately excluded through clinical, microscopic, and biopsy findings.

Dr. Catalina Matiz, a pediatric dermatologist at Southern California Permanente Medical Group, San Diego
Dr. Catalina Matiz


Management strategies for pediatric scalp DLE aim at preventing disease progression, minimizing scarring, and addressing aesthetic concerns. These include the use of topical and intralesional corticosteroids, calcineurin inhibitors, and antimalarial agents like hydroxychloroquine, alongside stringent photoprotection to mitigate UV-triggered exacerbations.
 

Conclusion

The prognosis for pediatric scalp DLE can be favorable with timely and appropriate management, underscoring the importance of early diagnosis and intervention to prevent scarring and hair loss. However, ongoing surveillance is crucial for monitoring potential progression to systemic lupus erythematosus, albeit a low-risk transformation.

Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego.

References

1. George PM and Tunnessen WW. Childhood discoid lupus erythematosus. Arch Dermatol. 1993;129(5):613-617.

2. Hawat T et al. Pediatric discoid lupus erythematosus: Short report. Dermatol Ther. 2022 Jan;35(1):e15170. doi: 10.1111/dth.15170.

3. Arkin LM et al. Practice-based differences in paediatric discoid lupus erythematosus. Br J Dermatol. 2019 Oct;181(4):805-810. doi: 10.1111/bjd.17780.

Publications
Publications
Topics
Article Type
Sections
Questionnaire Body

Patch of alopecia with some associated erythema and scale
A 16-year-old female was referred to the dermatology clinic for evaluation of multiple areas of hair loss on the scalp, which she described as occasionally itchy but not painful. The patient had not applied any treatments to these areas. Her medical history was notable for well-controlled eczema. She reported a family history of alopecia areata in her mother and mentioned occasional sun sensitivity. However, she denied experiencing joint pain, mouth sores, muscle weakness, or other systemic symptoms. Additionally, the patient was being treated for acne with adapalene and benzoyl peroxide. 
Upon physical examination, the patient exhibited several patches of alopecia with accompanying perifollicular scaling, crusting, and erythema on the affected areas. Examination of her face revealed comedones and papules.

Disallow All Ads
Content Gating
No Gating (article Unlocked/Free)
Alternative CME
Disqus Comments
Default
Use ProPublica
Hide sidebar & use full width
render the right sidebar.
Conference Recap Checkbox
Not Conference Recap
Clinical Edge
Display the Slideshow in this Article
Medscape Article
Display survey writer
Reuters content
Disable Inline Native ads
WebMD Article