Pityriasis rubra pilaris in children.

BACKGROUND

Pityriasis rubra pilaris (PRP) is an uncommon dermatosis in children. Few long-term studies on the treatment and prognosis of PRP in children have been performed.

OBJECTIVE

Our purpose was to retrospectively review the clinical course and treatment of all cases of PRP in children 19 years or younger who were seen at the Mayo Clinic.

METHODS

The clinical courses of the 30 patients with PRP seen at the Mayo Clinic between 1975 and 1997 were reviewed.

RESULTS

The most common presenting form of PRP in children is the type III juvenile form (Griffiths' criteria). Treatment ranged from topical steroids, tar, and ultraviolet B to systemic retinoids and methotrexate. The best response was obtained with isotretinoin; 5 of 6 patients showed 90% to 100% clearing within 6 months of treatment. Follow-up information was obtained by questionnaire and was available for 83% of patients. Overall, 43% had 90% to 100% resolution of their disease, 23% had a moderate response (30%-90% improvement), and 17% had a poor response (<30% improvement). One patient reported spontaneous resolution. Seventeen percent of those who had total clearing had recurrence of PRP within 1 year.

CONCLUSION

PRP in children is a noninherited dermatosis with no sex predilection, occurring mainly in the type III classic juvenile form. Retinoids should be considered as first-line treatment for PRP. Recurrence rate, previously thought to be rare, was about 17% in our population.