Penicillamine-induced toxic epidermal necrolysis in a patient with Wilson disease
Allergy, Asthma & Respiratory Disease
; : 302-305, 2014.
Article
em Ko
| WPRIM
| ID: wpr-29498
Biblioteca responsável:
WPRO
ABSTRACT
Toxic epidermal necrolysis (TEN) is rare but life-threatening severe cutaneous adverse reaction, which is mostly induced by drugs. It characterized by widespread epidermal necrosis, resulting in bullae with sloughing and frequent involvement of the mucous membrane. Due to high mortality, management of patients requires prompt withdrawal of the causative drug, appropriate supportive care, and consideration of immune-modulating agents, such as intravenous immunoglobulin or corticosteroids. Wilson disease is an inherited disorder of copper transport that results in excessive accumulation of copper in the body. Copper chelation with penicillamine is an effective first line therapy in most patients. We present a 20-year-old man with Wilson disease who developed TEN following administration of penicillamine. He was successfully treated with systemic corticosteroid, intravenous immunoglobulin, and supportive management.
Palavras-chave
Texto completo:
1
Base de dados:
WPRIM
Assunto principal:
Penicilamina
/
Imunoglobulinas
/
Mortalidade
/
Síndrome de Stevens-Johnson
/
Corticosteroides
/
Cobre
/
Degeneração Hepatolenticular
/
Mucosa
/
Necrose
Tipo de estudo:
Prognostic_studies
Limite:
Humans
Idioma:
Ko
Revista:
Allergy, Asthma & Respiratory Disease
Ano de publicação:
2014
Tipo de documento:
Article