Vesicular pemphigoid with circulating autoantibodies against 230-kDa and 180-kDa proteins, and additional autoantibodies against 97-kDa and 45-kDa proteins.
The vesicular variant of bullous pemphigoid is a clinical entity in which the principal type of lesions are multiple small tense vesicles in a symmetric distribution, instead of the classical large, more randomly distributed large bullae. We describe a 62-year-old female who developed a vesicular variant of bullous pemphigoid, with intensely pruritic vesiculopapular eruptions and erythematous irregularly outlined patches confluent on the face, neck, trunk, extremities and oral mucosa. Direct immunofluorescence revealed a linear deposition of IgG and C3 at the basement membrane zone of the skin, and indirect immunofluorescence detected circulating IgG autoantibodies reacting with antigens located on the epidermal side of skin split with 1 M NaCl. Indirect immunogold electron microscopy revealed IgG deposition at the underneath membrane of the basal cells and in the lamina lucida. In addition to the 230-kDa and 180-kDa bullous pemphigoid antigen, immunoblot analysis also demonstrated the presence of IgG antibodies reactive with 97-kDa and 45-kDa protein. Treatment with systemic corticosteroid, minocycline and antihistamines plus topical steroid resulted in moderate improvement. A few vesicles, however, continued to appear intermittently during the past year of treatment.
Lai
Feng-Jie
FJ
Department of Dermatology, National Cheng Kung University, Tainan, Taiwan.
Sheu
Hamm-Ming
HM
Lee
J Yu-Yun
JY
Cheng
Ching-Li
CL
Chen
Wenchieh
W
D001323:Autoantibodies / Q000097:blood*; D001324:Autoantigens / Q000097:blood / Q000276:immunology*; D002352:Carrier Proteins / Q000097:blood / Q000276:immunology*; D003598:Cytoskeletal Proteins / Q000097:blood / Q000276:immunology*; D004268:DNA-Binding Proteins; D000071821:Dystonin; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D015151:Immunoblotting; D008854:Microscopy, Electron; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000097:blood / Q000276:immunology*; D024041:Non-Fibrillar Collagens / Q000097:blood / Q000276:immunology*; D010391:Pemphigoid, Bullous / Q000097:blood / Q000276:immunology* / Q000473:pathology; D000097805:Collagen Type XVII
D001323:Autoantibodies; D001324:Autoantigens; D002352:Carrier Proteins; D003598:Cytoskeletal Proteins; D004268:DNA-Binding Proteins; D000071821:Dystonin; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII; C121408:DNA repair protein, 230-kDa, human; C486268:DST protein, human