A 2-year-old girl presented with a 2-week history of a rash, which started on her hands and feet and then spread all over her body after a few days. Her mother reported intermittent fever during the 2 weeks associated with irritability. Examination revealed bilateral small cervical lymph nodes and a widespread salmon pink macular rash with scaling plaques over her knees. Her palms and soles were thickened with fissuring and peeling of the skin (figures 1 and and2).2). A presumptive diagnosis of atypical Kawasaki disease was made and she was commenced on intravenous immunoglobulin. Dermatology consult was sought the next day, following which our diagnosis was revised to juvenile pityriasis rubra pilaris (PRP).
PRP is an uncommon disorder of keratinisation characterised by reddish-orange scaly plaques surrounded by islands of spared skin and marked thickening of the skin on the palms and soles. Onset can occur at any age, but is usually prepubertal or in the sixth decade.1 Diagnosis is based on clinical and histological findings. In children, the condition is usually self-limiting with 75% spontaneous resolution in 3–4 y. Treatment includes topical emollients and steroids. Systemic retinoids are useful in more severe disease.2 The cause of PRP is unknown. Most cases are sporadic or acquired following an acute infection. A rarer, familial, form is inherited in an autosomal dominant fashion. Fever is not a feature of PRP but an acute exanthematous form following an upper respiratory tract infection with features akin to Kawasaki’s disease has been described.3