Mucha-Habermann disease.
A case of Mucha-Habermann disease (MHD), possibly associated with macrophage activation syndrome (MAS), is reported. The purpose of this paper was to describe the rare MHD (also known as pityriasis lichenoides et varioliformis acuta - PLEVA) in a 28-year-old male, who presented with generalized ulceronecrotic lesions on the skin and mucosae, gastrointestinal involvement, and heart and liver failure, associated with continuous high fever.The patient might have progressed to MAS and eventually died. The MHD is rare, potentially fatal and has severe systemic complications.The importance of early diagnosis and aggressive treatment is emphasized.
Blanca Elena Rios Gomes
BE
Bica
Maria da Glória Costa Reis Monteiro
Mda G
de Barros
Carlos
C
Spingola
D000328:Adult; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D017514:Pityriasis Lichenoides / Q000175:diagnosis / Q000628:therapy