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

Journal: The Journal of dermatology 2004;31(1):10-5

PMID: 14739497

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Abstract

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.

Address: Departments of Dermatology, Osaka Red Cross Hospital, Ten'noji-ku, Osaka, Japan.

DOI: 10.1111/j.1346-8138.2004.tb00497.x

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MeSH Terms: Aged, Autoantibodies, Biopsy, Needle, Cell Adhesion Molecules, Drug Therapy, Combination, Fatal Outcome, Humans, Immunohistochemistry, Laryngoscopy, Laryngostenosis, Lung Neoplasms, Male, Pemphigoid, Benign Mucous Membrane, Risk Assessment, Severity of Illness Index, Tracheostomy, Kalinin