[Cicatricial pemphigoid with autoantibodies to laminin 5 (epiligrin) in a patient with metastatic endometrial carcinoma].
A 71-year-old female patient presented with erosions of the oral, genital and ocular mucosa, but without skin lesions. One year prior to the onset of lesions, a metastatic adenocarcinoma of the endometrium had been diagnosed. In perilesional skin, direct immunofluorescence showed linear deposits of C3 and IgG along the basement membrane zone. Indirect immunofluorescence demonstrated anti-basement membrane zone antibodies which bound exclusively to the dermal side of 1M NaCl-split skin. In immunoprecipitation studies, the autoantibodies bound a set of proteins characteristic of epiligrin/laminin-5, and they specifically immunoblotted the alpha-subunit of this protein. These results confirmed our presumed diagnosis of anti-epiligrin cicatricial pemphigoid. Combined treatment with methylprednisolone and dapsone resulted in prompt remission, despite progression of her malignancy. So far, only eight patients with anti-epiligrin cicatricial pemphigoid have been reported worldwide. Our patient represents the first case from Austria and is clinically and immunopathologically similar to previously reported cases.
Lenz
P
P
Abteilung für Immundermatologie und infektiöse Hautkrankheiten, Universitätsklinik für Dermatologie, Allgemeines Krankenhaus der Stadt Wien, Osterreich.
Hsu
R
R
Yee
C
C
Yancey
K
K
Volc-Platzer
B
B
Stingl
G
G
Kirnbauer
R
R
D000230:Adenocarcinoma / Q000175:diagnosis / Q000276:immunology* / Q000473:pathology; D000368:Aged; D001323:Autoantibodies / Q000097:blood*; D001485:Basement Membrane / Q000276:immunology / Q000473:pathology; D015815:Cell Adhesion Molecules / Q000276:immunology*; D016889:Endometrial Neoplasms / Q000175:diagnosis / Q000276:immunology* / Q000473:pathology; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D005500:Follow-Up Studies; D006801:Humans; D010257:Paraneoplastic Syndromes / Q000175:diagnosis / Q000276:immunology* / Q000473:pathology; D010390:Pemphigoid, Benign Mucous Membrane / Q000175:diagnosis / Q000276:immunology* / Q000473:pathology; D000097794:Kalinin
D001323:Autoantibodies; D015815:Cell Adhesion Molecules; D000097794:Kalinin
Vernarbendes Pemphigoid mit Autoantikörpern gegen Laminin-5 (Epiligrin) bei einer Patientin mit metastasierendem Endometriumkarzinom.