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Lawrence S. Chan, MD; Jean-Christophe Lapiere, MD; Mei Chen, PhD; Tom Traczyk, BS; Anthony J. Mancini, MD; Amy S. Paller, MD; David T. Woodley, MD; M. Peter Marinkovich, MD

Arch Dermatol. 1999;135:569-573.

Background  Bullous systemic lupus erythematosus is a generalized subepidermal blistering skin eruption in patients suffering from systemic lupus erythematosus. Type VII collagen was initially identified as the target antigen.

Observation  We studied an unusual patient who had bullous systemic lupus erythematosus. The patient fulfilled the criteria of systemic lupus with an antinuclear antibody titer of 1:5120. Immunopathological testing revealed in vivo deposition of all IgG subclasses, secretory IgA1, and both light chains at the patient's skin basement membrane. The in vivo–bound IgG and IgA were localized at the hemidesmosomes and lamina densa. The patient's IgG and IgA circulating autoantibodies labeled both the epidermal roof and the dermal floor of salt-split skin and recognized the hemidesmosomal protein BP230 as well as the full-length native form and the recombinant noncollagenous domain 1 of type VII collagen (anchoring fibril). In addition, the patient's IgG autoantibodies recognized the anchoring filament proteins laminin-5 and laminin-6 (3 chain and 2 chain).

Conclusions  We conclude that patients with bullous systemic lupus erythematosus may have autoantibodies to multiple basement membrane components critical for epidermal-dermal junctional adhesion. Possible pathogenic mechanisms in this patient's clinical diseases include provocation of organ-specific disease (bullous disease) by systemic autoimmunity (lupus) and the "epitope spreading" immune phenomenon.

From the Medicine Service, Section of Dermatology, Lakeside Division, Veterans Affairs Chicago Health Care System (Dr Chan), and the Departments of Dermatology (Drs Chan, Lapiere, Chen, Mancini, Paller, and Woodley and Mr Traczyk) and Pediatrics (Drs Mancini and Paller), Northwestern University Medical School, Chicago, Ill; the Department of Dermatology, Stanford University School of Medicine, Stanford (Dr Marinkovich), and the Dermatology Service, Palo Alto Veterans Affairs Health Care System, Palo Alto (Dr Marinkovich), Calif.

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