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

Allergy Asthma & Respiratory Disease 2014³â 2±Ç 4È£ p.302 ~ p.305

ÀÌÀ±ÀÓ(Lee Yoon-Im) - ¿ï»ê´ëÇб³ Àǰú´ëÇÐ ¿ï»ê´ëÇб³º´¿ø ³»°ú
À̹«¿­(Lee Mu-Yeol) - ¿ï»ê´ëÇб³ Àǰú´ëÇÐ ¿ï»ê´ëÇб³º´¿ø ³»°ú
¹Ú¿ëÅÂ(Park Yong-Tae) - ¿ï»ê´ëÇб³ Àǰú´ëÇÐ ¿ï»ê´ëÇб³º´¿ø ³»°ú
¹ÚÂùÈ£(Park Chan-Ho) - ¿ï»ê´ëÇб³ Àǰú´ëÇÐ ¿ï»ê´ëÇб³º´¿ø ³»°ú
¼ºÁØ¿µ(Seong Jun-Young) - ¿ï»ê´ëÇб³ Àǰú´ëÇÐ ¿ï»ê´ëÇб³º´¿ø ÇǺΰúÇб³½Ç
¼­È£¼®(Suh Ho-Seok) - ¿ï»ê´ëÇб³ Àǰú´ëÇÐ ¿ï»ê´ëÇб³º´¿ø ÇǺΰúÇб³½Ç
Ãֽ¿ø(Choi Seung-Won) - ¿ï»ê´ëÇб³ Àǰú´ëÇÐ ¿ï»ê´ëÇб³º´¿ø ³»°ú

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.

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Toxic epidermal necrolysis, Stevens-Johnson syndrome, Penicillamine
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