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Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus and the spectrum of the acute syndrome of apoptotic pan-epidermolysis (ASAP): a case report, concept review and proposal for new classification of lupus erythematosus vesiculobullous skin lesions

Department of Dermatology Camino Medical Group, Sunnyvale, CA, USA

M S Stone

Department of Dermatology and Pathology, University of Iowa, Roy J and Lucille A Carver College of Medicine, IA, USA

D Racila

Department of Dermatology and Pathology, University of Iowa, Roy J and Lucille A Carver College of Medicine, IA, USA

R H Scofield

Department of Medicine, University of Oklahoma Health Science Center, Arthritis and Immunology Program, Oklahoma Medical Research Foundation, OK, USA

R D Sontheimer

Department of Dermatology and Pathology, University of Iowa, Roy J and Lucille A Carver College of Medicine, IA, USA, richard-sontheimer{at}uiowa.edu

The acute clinical syndrome of toxic epidermal necrolysis (TEN) is currently thought to be a distinct clinical-pathological entity typically resulting from drug hypersensitivity. We describe an adult woman who experienced a fulminate pattern of apoptotic epidermal cell injury following tanning bed exposure while taking naproxen that resulted in a clinical presentation having combined features of drug-induced TEN and an infrequently recognized form of bullous cutaneous lupus erythematosus (LE). This case calls attention to the fact that TEN-like injury can occasionally be seen in settings other than drug hypersensitivity (e.g., LE, acute graft versus host disease) and illustrates the need for a unifying concept in this area. We therefore propose the term ‘Acute Syndrome of Apoptotic Pan-Epidermolysis (ASAP)’ to designate a clinical syndrome that is characterized by life-threatening acute and massive cleavage of the epidermis resulting from hyperacute apoptotic injury of the epidermis. We also review vesiculobullous skin disorders that can be encountered in LE patients and suggest a new classification scheme for such lesions.

Key Words: cutaneous lupus erythematosus • graft-versus-host disease • toxic epidermal necrolysis

Lupus, Vol. 13, No. 12, 941-950 (2004)
DOI: 10.1191/0961203304lu2037sa


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