Korean J Dermatol.  2012 Oct;50(10):912-916.

Anti-p200 Pemphigoid

• Lee MH
• Yoo JY
• Suh MK
• Jang TJ
• Kim SC

Abstract

Anti-p200 pemphigoid is an autoimmune subepidermal bullous disease characterized by circulating and tissue-bound autoantibodies directed against a 200 kD protein of the human dermis. We report a 78-year-old male who was presented with bullous eruptions on the trunk, extremities, which clinically resemble bullous pemphigoid, epidermolysis bullosa aquisita, linear IgA dermatosis or dermatitis herpetiformis. Oral muscosa of the lower lip was also affected. Histopathological examination of a skin biopsy specimen from the trunk revealed subepidermal blister and infiltration predominantly by neutrophils. Direct immunofluorescence revealed linear deposits of IgG, C3 and IgA at the basement membrane zone. Indirect immunofluorescence using salt-split skin showed that IgG antibodies bound on the dermal side. Immunoblotting with dermal extracts showed that the patient's IgG autoantibodies reacted with a 200 kD protein. The patient showed good response to dapsone.

Keyword

Anti-p200 pemphigoid; 200 kD antigen

MeSH Terms

Aged
Antibodies
Autoantibodies
Basement Membrane
Biopsy
Blister
Dapsone
Dermatitis Herpetiformis
Dermis
Epidermolysis Bullosa
Extremities
Fluorescent Antibody Technique, Direct
Fluorescent Antibody Technique, Indirect
Humans
Immunoblotting
Immunoglobulin A
Immunoglobulin G
Linear IgA Bullous Dermatosis
Lip
Male
Neutrophils
Pemphigoid, Bullous
Skin
Antibodies
Autoantibodies
Dapsone
Immunoglobulin A
Immunoglobulin G