Doença de Mucha-Habermann / Mucha-Habermann disease
Rev. bras. reumatol
; 53(3): 314-317, maio-jun. 2013. ilus
Article
in Portuguese
| LILACS
| ID: lil-686094
Responsible library:
BR1.1
RESUMO
Os autores descrevem um caso de doença de Mucha-Habermann (DMH), que cursou com quadro sugestivo de síndrome de ativação macrofágica (SAM). O objetivo do trabalho foi descrever um caso de rara vasculite de Mucha-Habermann (pitiríase liquenoide e varioliforme aguda - PLEVA) em paciente de 28 anos que apresentou lesões ulceronecróticas generalizadas em pele e mucosas, acometimento gastrointestinal, cardíaco e hepático, associados a febre alta contínua, com provável evolução para SAM e posterior óbito. Trata-se de doença rara, potencialmente fatal, com graves complicações sistêmicas. Os autores ressaltam a importância de seu diagnóstico e de tratamento agressivo.
ABSTRACT
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 andliver 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.
Full text:
Available
Collection:
International databases
Database:
LILACS
Main subject:
Pityriasis Lichenoides
Type of study:
Diagnostic study
/
Screening study
Limits:
Adult
/
Humans
/
Male
Language:
Portuguese
Journal:
Rev. bras. reumatol
Journal subject:
Rheumatology
Year:
2013
Document type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
Universidade Federal do Rio de Janeiro/BR