Penicillamine-induced toxic epidermal necrolysis in a patient with Wilson disease

Yoon Im Lee; Mu Yeol Lee; Yong Tae Park; Chan Ho Park; Jun Young Sung; Ho Seok Seo; Seung Won Choi

doi:10.4168/aard.2014.2.4.302

Journal List > Allergy Asthma Respir Dis > v.2(4) > 1059046

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Lee, Lee, Park, Park, Sung, Seo, and Choi: Penicillamine-induced toxic epidermal necrolysis in a patient with Wilson disease

Case Report

Allergy, Asthma & Respiratory Disease 2014; 2(4): 302-305.

Published online: 30 September 2014

DOI: https://doi.org/10.4168/aard.2014.2.4.302

Penicillamine-induced toxic epidermal necrolysis in a patient with Wilson disease

Yoon Im Lee¹, Mu Yeol Lee¹, Yong Tae Park¹, Chan Ho Park¹, Jun Young Sung², Ho Seok Seo², Seung Won Choi¹

¹Department of Internal Medicine, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea.

²Department of Dermatology, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea.

Correspondence to: Seung Won Choi. Department of Internal Medicine, Ulsan University Hospital, University of Ulsan College of Medicine, 877 Bangeojinsunhwan-doro, Dong-gu, Ulsan 682-714, Korea. Tel: +82-52-250-7029, Fax: +82-52-250-7048, choisw@uuh.ulsan.kr

Received 15 January 2014 Revised 26 February 2014 Accepted 13 March 2014

(open-access):

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/).

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.

Keywords: Toxic epidermal necrolysis, Stevens-Johnson syndrome, Penicillamine

Figures and Tables

Fig. 1

Widespread erythematous maculopapular eruption with bullae and epidermal detachment are showing on the back and neck.

Fig. 2

Skin biopsy specimen reveals epidermal necrosis, necrotic keratinocytes, and subepidermal bullae with epidermal detachment from dermis (H&E, ×400).

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