Penicillamine-induced toxic epidermal necrolysis in a patient with Wilson disease.
10.4168/aard.2014.2.4.302
- Author:
Yoon Im LEE
1
;
Mu Yeol LEE
;
Yong Tae PARK
;
Chan Ho PARK
;
Jun Young SUNG
;
Ho Seok SEO
;
Seung Won CHOI
Author Information
1. Department of Internal Medicine, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea. choisw@uuh.ulsan.kr
- Publication Type:Case Report
- Keywords:
Toxic epidermal necrolysis;
Stevens-Johnson syndrome;
Penicillamine
- MeSH:
Adrenal Cortex Hormones;
Copper;
Hepatolenticular Degeneration*;
Humans;
Immunoglobulins;
Mortality;
Mucous Membrane;
Necrosis;
Penicillamine;
Stevens-Johnson Syndrome*;
Young Adult
- From:Allergy, Asthma & Respiratory Disease
2014;2(4):302-305
- CountryRepublic of Korea
- Language:Korean
-
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.
