Vernarbendes pemphigoid mit autoantikorpern gegen laminin-5 (epiligrin) bei einer patientin mit metastasierendem endometriumkarzinom

Translated title of the contribution: Cicatricial pemphigoid with anti-epiligrin autoantibodies in a patient with metastatic adenocarcinoma of the endometrium

P. Lenz, R. Hsu, C. Yee, Kim Yancey, B. Volc-Platzer, G. Stingl, R. Kirnbauer

Research output: Contribution to journalArticlepeer-review

23 Scopus citations

Abstract

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 dereal side of 1 M 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.

Translated title of the contributionCicatricial pemphigoid with anti-epiligrin autoantibodies in a patient with metastatic adenocarcinoma of the endometrium
Original languageGerman
Pages (from-to)31-35
Number of pages5
JournalHautarzt
Volume49
Issue number1
DOIs
StatePublished - Jan 1 1998

Keywords

  • Anti-epiligrin autoantibodies
  • Cicatricial pemphigoid
  • Immunosuppressive therapy
  • Paraneoplasia

ASJC Scopus subject areas

  • Dermatology

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