Doença de Mucha-Habermann (variante febril úlcero-necrótica) com acometimento mucoso exuberante: relato de caso

Pityriasis lichenoides is a rare idiopathic cutaneous disorder, with a clinical-histopathological spectrum comprising the acute varioliform form (Mucha-Habermann's disease), its febrile ulceronecrotic variant, and its chronic form. Systemic manifestations may occur in the febrile ulceronecrotic variant, with reports of adult mortality. The case of a young male patient with clinical and histopathological diagnosis of Mucha-Habermann’s disease, febrile ulceronecrotic variant, with severe mucosal involvement - an occasional incidence even in the most severe forms of pityriasis lichenoides - is presented. In addition to the atypical clinical aspect, an excellent therapeutic result is shown with the association of prednisone and methotrexate.