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

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 e...

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Bibliographic Details
Published in:Lupus 2004-01, Vol.13 (12), p.941-950
Main Authors: Ting, W, Stone, M S, Racila, D, Scofield, R H, Sontheimer, R D
Format: Article
Language:English
Subjects:
Acute Disease
Diagnosis, Differential
Female
Humans
Lupus Erythematosus, Cutaneous - classification
Lupus Erythematosus, Cutaneous - complications
Lupus Erythematosus, Cutaneous - diagnosis
Middle Aged
Skin Diseases, Vesiculobullous - classification
Skin Diseases, Vesiculobullous - diagnosis
Skin Diseases, Vesiculobullous - etiology
Stevens-Johnson Syndrome - diagnosis
Stevens-Johnson Syndrome - etiology
Syndrome
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Summary: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.
ISSN:0961-2033
1477-0962
DOI:10.1191/0961203304lu2037sa