Bullous systemic lupus erythematosus with autoantibodies recognizing multiple skin basement membrane components, bullous pemphigoid antigen 1, laminin-5, laminin-6, and type VII collagen

Lawrence S. Chan*, Jean Christophe Lapiere, Mei Chen, Tom Traczyk, Anthony J. Mancini, Amy S. Paller, David T. Woodley, M. Peter Marinkovich

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

96 Scopus citations

Abstract

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 (α3 chain and γ2 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.

Original languageEnglish (US)
Pages (from-to)569-573
Number of pages5
JournalArchives of dermatology
Volume135
Issue number5
DOIs
StatePublished - May 1999

ASJC Scopus subject areas

  • Dermatology

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