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

Research output: Contribution to journalArticle

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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 - 1998

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Epidermolysis Bullosa Acquisita
Benign Mucous Membrane Pemphigoid
Stomach
Carcinoma
Skin
Blister
Fluorescence Microscopy
Autoantibodies
Immunoglobulin G
Direct Fluorescent Antibody Technique
Immunoelectron Microscopy
Laminin
Gastrectomy
Indirect Fluorescent Antibody Technique
Serum
Keratinocytes
Basement Membrane
Protein Isoforms
Adenocarcinoma
Lymph Nodes

ASJC Scopus subject areas

  • Dermatology

Cite this

Anti-epiligrin cicatricial pemphigoid : A case associated with gastric carcinoma and features resembling epidermolysis bullosa acquisita. / Fujimoto, W.; Ishida-Yamamoto, A.; Hsu, R.; Nagao, Y.; Hzuka, H.; Yancey, K. B.; Arata, J.

In: British Journal of Dermatology, Vol. 139, No. 4, 1998, p. 682-687.

Research output: Contribution to journalArticle

Fujimoto, W. ; Ishida-Yamamoto, A. ; Hsu, R. ; Nagao, Y. ; Hzuka, H. ; Yancey, K. B. ; Arata, J. / Anti-epiligrin cicatricial pemphigoid : A case associated with gastric carcinoma and features resembling epidermolysis bullosa acquisita. In: British Journal of Dermatology. 1998 ; Vol. 139, No. 4. pp. 682-687.
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