A Case of Anti-Epiligrin Cicatricial Pemphigoid Associated with Lung Carcinoma and Severe Laryngeal Stenosis: Review of Japanese Cases and Evaluation of Risk for Internal Malignancy

Satoko Matsushima, Yuji Horiguchi, Tetsuya Honda, Shinobu Fujii, Takayuki Okano, Masahiro Tanabe, Toshiaki Wakayama, Takashi Hashimoto, Kim B. Yancey

Research output: Contribution to journalReview article

26 Scopus citations


A 68-year-old Japanese male with a five-year-history of lung carcinoma showed recurrent blisters and erosions on the oral and genital mucosae and the skin. The patient complained of dyspnea due to severe laryngeal stenosis and underwent a tracheostomy. A skin biopsy specimen showed a subepidermal blister and linear deposits of IgG and C3 at the basement membrane zone of the epidermis. Indirect immunofluorescence examination demonstrated circulating IgG anti-basement membrane zone autoantibodies that reacted to epiligrin on immunoblotting. Based on a diagnosis of anti-epiligrin cicatricial pemphigoid, he was treated with prednisolone, minocycline hydrochloride and nicotinamide. Although no new skin lesions appeared, he died of lung carcinoma five months after the tracheostomy. A review of reported cases with anti-epiligrin cicatricial pemphigoid in Japan disclosed that 5 of 16 cases (31.2%) were complicated by internal malignancies.

Original languageEnglish (US)
Pages (from-to)10-15
Number of pages6
JournalJournal of Dermatology
Issue number1
StatePublished - Jan 2004



  • Anti-epiligrin cicatricial pemphigoid
  • Internal malignancies
  • Laryngeal stenosis

ASJC Scopus subject areas

  • Dermatology

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