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

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 crythematosus. 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 (alpha3 chain and gamma2 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.

• Authors: L S Chan, J C Lapiere, M Chen, T Traczyk, A J Mancini, A S Paller, D T Woodley, M P Marinkovich
• Journal: Archives of dermatology (Arch Dermatol) Vol. 135 Issue 5 Pg. 569-73 (May 1999) ISSN: 0003-987X [Print] United States
• PMID: 10328198 (Publication Type: Case Reports, Journal Article, Research Support, U.S. Gov't, Non-P.H.S., Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Autoantibodies
• Autoantigens
• Carrier Proteins
• Cell Adhesion Molecules
• Cytoskeletal Proteins
• DST protein, human
• Dystonin
• Laminin
• Nerve Tissue Proteins
• Non-Fibrillar Collagens
• collagen type XVII
• kalinin
• laminin 6
• Collagen

Topics

• Adolescent
• Autoantibodies (immunology)
• Autoantigens (immunology)
• Basement Membrane (immunology)
• Carrier Proteins
• Cell Adhesion Molecules (immunology)
• Collagen (immunology)
• Cytoskeletal Proteins
• Dystonin
• Female
• Humans
• Laminin (immunology)
• Lupus Erythematosus, Systemic (complications, immunology)
• Nerve Tissue Proteins
• Non-Fibrillar Collagens
• Pemphigoid, Bullous (complications, immunology)