Catalog

Pityriasis rubra pilaris type I, classical adult

No MMS code

Link to WHO Definition

Pityriasis rubra pilaris type I, classical adult – http://id.who.int/icd/entity/1296778126

This is the most common and recognisable form of pityriasis rubra pilaris. It presents as a rapidly evolving scaly erythematous rash which is initially follicular but typically progresses to erythroderma, within which small islands of uninvolved skin characteristically remain. Untreated, it tends to resolve spontaneously after an average of three years. It affects the sexes equally with the highest incidence between 40 and 60 years of age. The cause is poorly understood though gain-of-function mutations in the CARD14 gene have been linked to rare genetic forms of the disease.