Alterações esclerodermiformes nas mãos de uma adolescente

Objective Illustrate an typical presentation of a rare dermatosis. Methods Pityriasis rubra pilaris (PRP) is a rare inflammatory papulo-squamous dermatosis. Genetic factors, changes in vitamin A metabolism and infections have been proposed as triggering mechanism of the disease, but its etiology and pathogenesis are still unknown. It has been described in association with HIV, drugs, autoimmune disease and visceral neoplasms. It affects all races and genders equally, with a peak distribution in the first and fifth decades of life. Most case occur sporadically, but familial cases have been reported. Results Adolescent, 18 years old, with report of generalizes pruritic skin lesions since the age of 3 months, treatd only with antihistamines. He denied comorbidities, allergies or family history of skin diseases. On examination, follicular hyperkeratosis alternating with islands of healthy skin on the anterior and posterior trunk, ichthyosiform desquamation on the lower limbs, palmoplantar keratoderma and sclerodermiform alterations on the hands and feet were noted. A biposy of the lesion on the back was performed, which showed psoriasiform dermatitis with orhokeratosis and parakeratosis alternating horizontally. Considering the clinical-pathological correlation, the diagnosis was juvenile atypical PRP subtype. Conclusion The juvenile atypical subtype of PRP is the least frequent (5% of cases) and accounts for most familial cases. It appears in the first years of life and has a chronic course. It is characterized by follicular hyperkeratosis, erythema, ichthyosiform desquamation on the lower limbs, gross palmoplantar keratoderma and sclerodermiform alterations of the hands and feet.