Trimethoprim-sulfamethoxazole-induced linear IgA bullous disease presenting as toxic epidermal necrolysis.

BACKGROUND

Linear IgA bullous dermatosis (LABD) is an autoimmune blistering skin disorder characterized by linear IgA deposits along the dermoepidermal junction. Usually idiopathic, LABD can be drug-induced.

OBJECTIVE

To report the atypical characteristics of a case of trimethoprim-sulfamethoxazole-induced LABD presenting as toxic epidermal necrolysis (TEN).

METHODS

A 63-year-old woman treated with trimethoprim-sulfamethoxazole for Pneumocystis jirovecii infection developed a generalized maculopapular rash with herpetiform lesions, rosette-like lesions, and tense bullae with Nikolsky sign.

RESULTS

Anti-basement membrane zone antibodies were negative, but immunoblot revealed a 160 kDa band corresponding to subepidermal class IgA desmoglein 1. Skin biopsy specimens revealed a subepidermal bulla and direct immunofluorescence showed linear IgA deposition along the basement membrane zone. A diagnosis of toxic epidermal necrolysis was excluded and replaced by trimethoprim-sulfamethoxazole-induced LABD.

CONCLUSION

We report a case of trimethoprim-sulfamethoxazole-induced LABD with a 160 kDa IgA desmoglein 1 found by immunoblotting analysis, probably by epitope spreading.