Circumscribed juvenile pityriasis rubra pilaris

A 7-year-old girl presented with well-demarcated erythematous, scaly hyperkeratotic plaques, bilaterally symmetrically on her elbows and knees [Figure 1a], along with diffuse waxy hyperkeratosis over the palmoplantar region [Figure 1b] for the past 9 months. Biopsy from right knee area revealed alternating parakeratosis and orthokeratosis, sparse lymphohistiocytic perivascular infiltrate in the dermis, consistent with pityriasis rubra pilaris in agreement with our clinical diagnosis of circumscribed pityriasis rubra pilaris. Once daily application of topical betamethasone dipropionate 0.05% cream over the lesions was advised to the patient. After 2 weeks, improvement was noted.

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Figure 1:

(a) Well-demarcated erythematous, scaly hyperkeratotic plaques, bilaterally symmetrically on her elbows and knees and (b) Bilateral diffuse palmoplantar keratoderma.

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In children, the circumscribed juvenile subtype is the most common form of pityriasis rubra pilaris. It is frequently mistaken for other skin diseases such as psoriasis and atopic dermatitis.

Topical medications such as corticosteroids, tretinoin, and keratolytic are the initial treatment of choice, while systemic therapy is reserved for recalcitrant cases.^[1]