Pityriasis rubra pilaris in children

Juvenile pityriasis rubra pilaris: Report of 28 cases in Taiwan

“…10 Type V PRP is rare and absent in reports of large series, although many sporadic cases have been described in the recent literature. 4,5,8,[10][11][12][13][14][15] Piamphongsant and Akaraphant 5 from Thailand reported a total of 104 patients with juvenile PRP confirmed by clinical diagnosis without histopathology. The majority of their patients (100 of 104, 96.2%) had focal involvement: 21 of 100 had only palmoplantar hyperkeratosis, 59 of 100 had palmoplantar hyperkeratosis and circumscribed lesions on the elbows and knees, and 20 of 100 had palmoplantar hyperkeratosis and small scaly patches on the face and trunk, whereas only 4 juvenile patients (4 of 104, 3.8%) had generalized involvement.…”

“…[24][25][26][27][28][29] Systemic retinoids have been demonstrated to yield excellent therapeutic results. 8,12,[30][31][32] In the study by Dicken, 30 to 80 years with isotretinoin (1-2 mg/kg/d); the response rate increased to 100% when treatment was continued for up to 16 weeks. In the series of Allison et al, 8 5 of 6 patients with juvenile PRP had an excellent response to treatment with isotretinoin (0.75-1.5 mg/kg/d) for less than 6 months.…”

“…8 The response to each administered treatment was analyzed by measuring the rise and fall of disease severity according to the duration and dosage of the medication, with special attention to the use of systemic retinoids.…”

“…6,7 Because of the rarity of the disease, especially in children, large-scale studies of juvenile PRP are limited with diverse conclusions about its manifestation, classification, and treatment. 4,8 Here we report a long-term observational study of 28 juvenile cases in Taiwan.…”

Peripheral ulcerative keratitis in pityriasis rubra pilaris

“…The skin, nails, mucous membranes, and eyes can be affected. 1 Histopathology shows follicular plugging, acanthosis, hyperkeratosis, and a lymphohistiocytic dermal infiltrate. 2 Treatment includes emollients, systemic steroids, retinoids, and immunosuppressants.…”