A clinical case of pityriasis rubra pilaris - juvenile type

Abstract

Pityriasis rubra pilaris is a rare chronic inflammatory papulo-squamous skin disease, the pathogenesis of which is still unclear. The pathological essence of the disease is associated with a violation of keratinization against the background of hyperactivity of keratinocytes with subsequent inflammation, as well as with vitamin A dysmetabolism and a weakening of the protein-binding fiction of the liver. Unfortunately, treatment is complex with inconsistent improvement from topical therapies, including emollients, keratolytics, corticosteroids, vitamin D analogs, and retinoids. We present a clinical case of the juvenile type of Devergie’s disease in a 3-year-old child, which was caused by poisoning.

Keywords

• Pityriasis rubra pilaris
• Devergie's disease
• juvenile form
• systemic retinoids