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Juvenile Pityriasis Rubra Pilaris

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Case Presentation

A 4-year-old girl, without a prior history of eczema or psoriasis, presented with a 4-weeks history of extensive erythematous scaly eruption which started on her face and scalp, and spread rapidly to her torso and limbs ( Figure 1, A, B, E and F ). She also had well-circumscribed, red-orange, waxy plaques on the palms and soles ( Figure 1, C and D ). Her growth and psychomotor development were normal. Histopathology showed basket-like hyperkeratosis, diffuse orthokeratosis and spotted parakeratosis, and a dermal superficial perivascular lymphocytic infiltration ( Figure 1G ). The final diagnosis was pityriasis rubra pilaris.

Figure 1 .

(A,B,E,F) Extensive erythematous scaly eruption on the trunk and limbs. (C,D) Well-circumscribed, red-orange, waxy plaques on the patient palms and soles. (G) Basket-like hyperkeratosis, diffuse orthokeratosis and spotted parakeratosis, and a dermal superficial perivascular lymphocytic infiltration (H&E ×400).

Teaching Point

Pityriasis rubra pilaris is a rare inflammatory skin disease that affects men and women of all ages, which presents with hyperkeratotic follicular papules, erythematous-desquamative plaques, palmoplantar keratoderma [ 1 ] . Its classification into five subgroups is based on age at onset, clinical course, morphologic features, and prognosis [ 2 ] . We present a type III pityriasis rubra pilaris. The differential diagnosis includes psoriasis vulgaris, perifollicular keratosis, lichen spinulosus.

References

  1. A Review on Pityriasis Rubra Pilaris Wang D, Chong VC, Chong WS, Oon HH. Am J Clin Dermatol.2018;19(3):377-390. CrossRef PubMed
  2. Pityriasis rubra pilaris: a review of diagnosis and treatment Cohen PR, Prystowsky JH. J Am Acad Dermatol.1989;20(5 Pt 1):801-807. CrossRef PubMed

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