Anti-epiligrin cicatricial pemphigoid: A case associated with gastric carcinoma and features resembling epidermolysis bullosa acquisita

W. Fujimoto, A. Ishida-Yamamoto, R. Hsu, Y. Nagao, H. Hzuka, K. B. Yancey, J. Arata

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38 Scopus citations

Abstract

A 48-year-old woman with anti-epiligrin cicatricial pemphigoid (CP) who showed clinical features resembling epidermolysis bullosa acquisita was found to have adenocarcinoma of the stomach. Histological examination of lesional skin demonstrated a subepidermal blister. Direct immunofluorescence microscopy of perilesional skin revealed linear deposits of IgG and C3 at the basement membrane zone. The patient's serum contained IgG autoantibodies that bound to the dermal side of 1 mol/L NaCl-split normal human skin as determined by indirect immunofluorescence microscopy, and the lamina lucida as determined by indirect immunoelectron microscopy. The patient's serum immunoprecipitated laminin-5 from extracts and media of biosynthetically radiolabelled human keratinocytes. Immunoblot studies showed that the patient's autoantibodies specifically bound the α3 subunit of this laminin isoform. Fragility of the skin and bullous lesions disappeared after total gastrectomy, but soon reappeared possibly in association with metastatic disease in a lymph node. The possibility that anti-epiligrin CP may develop paraneoplastically in some patients is discussed.

Original languageEnglish (US)
Pages (from-to)682-687
Number of pages6
JournalBritish Journal of Dermatology
Volume139
Issue number4
StatePublished - Oct 27 1998

ASJC Scopus subject areas

  • Dermatology

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