Usefulness of a Simple Immunohistochemical Staining Technique to Differentiate Anti-p200 Pemphigoid From Other Autoimmune Blistering Diseases: A Report of 2 Cases.
Anti-p200 pemphigoid is a rare autoimmune subepidermal blistering disease characterized by the presence of circulating immunoglobulin G antibodies directed against laminin gamma-1, a 200-kDa protein located in the lamina lucida of the basement membrane. We review the clinical, histopathological and immunological characteristics of the first 2 cases described in Spain. Anti-p200 pemphigoid shares histopathological and immunopathological findings with epidermolysis bullosa acquisita, the main entity in the differential diagnosis. However, its management follows the same guidelines as those used for bullous pemphigoid. The diagnosis is confirmed by immunoblotting, which is a complex technique available in few centers. We propose the immunohistochemical detection of collagen type IV on the floor of the blister, combined with standard immunofluorescence techniques, as a simple, accessible alternative to differentiate anti-p200 pemphigoid from epidermolysis bullosa acquisita.
Departamento de Dermatología, Hospital del Mar, Parc de Salut Mar, Institut Hospital del Mar d'Investigacions Mèdiques, Barcelona, España. Electronic address: 60495@parcdesalutmar.cat.
I
I
García-Díez
Departamento de Dermatología, Hospital del Mar, Parc de Salut Mar, Institut Hospital del Mar d'Investigacions Mèdiques, Barcelona, España.
M E
ME
Martínez-Escala
Departamento de Dermatología, Facultad de Medicina de la Universidad de Kurume, Fukuoka, Japón.
N
N
Ishii
Departamento de Dermatología, Facultad de Medicina de la Universidad de Kurume, Fukuoka, Japón.
T
T
Hashimoto
Departamento de Dermatología, Hospital Clínic, Barcelona, España.
J M
JM
Mascaró Galy
Departamento de Dermatología, Hospital del Mar, Parc de Salut Mar, Institut Hospital del Mar d'Investigacions Mèdiques, Barcelona, España.
R M
RM
Pujol
Departamento de Dermatología, Hospital del Mar, Parc de Salut Mar, Institut Hospital del Mar d'Investigacions Mèdiques, Barcelona, España.
J E
JE
Herrero-González
D000328:Adult; D001323:Autoantibodies / Q000032:analysis*; D001324:Autoantigens / Q000276:immunology*; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000378:metabolism; D001768:Blister / Q000175:diagnosis / Q000378:metabolism; D024141:Collagen Type IV / Q000032:analysis*; D003176:Complement C3 / Q000032:analysis; D003622:Dapsone / Q000627:therapeutic use; D003937:Diagnosis, Differential; D016107:Epidermolysis Bullosa Acquisita / Q000175:diagnosis / Q000378:metabolism; D019085:Fluorescent Antibody Technique, Direct; D019084:Fluorescent Antibody Technique, Indirect; D013700:Giant Cell Arteritis / Q000150:complications / Q000188:drug therapy; D006801:Humans; D015151:Immunoblotting; D007074:Immunoglobulin G / Q000032:analysis*; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D007797:Laminin / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D008970:Molecular Weight; D010391:Pemphigoid, Bullous / Q000175:diagnosis* / Q000276:immunology / Q000378:metabolism; D011241:Prednisone / Q000009:adverse effects / Q000627:therapeutic use; D012872:Skin Diseases, Vesiculobullous / Q000175:diagnosis / Q000378:metabolism; D013194:Staining and Labeling / Q000379:methods*
D001323:Autoantibodies; D001324:Autoantigens; D024141:Collagen Type IV; D003176:Complement C3; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D007797:Laminin; C089141:laminin gamma 1; D003622:Dapsone; D011241:Prednisone
Anti-p200 pemphigoid; Bullous skin diseases; Collagen type IV; Colágeno tipo iv; Enfermedades ampollosas; Immunoblotting; Inmunoblot; Laminin gamma 1; Laminina gamma-1; Penfigoide anti-p200