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

• Published in: Journal of dermatology Vol. 31; no. 1; pp. 10 - 15
• Main Authors: Matsushima, Satoko, Horiguchi, Yuji, Honda, Tetsuya, Fujii, Shinobu, Okano, Takayuki, Tanabe, Masahiro, Wakayama, Toshiaki, Hashimoto, Takashi, Yancey, Kim B.
• Format: Journal Article
• Language: English
• Published: England 01-01-2004
• Subjects: Aged; anti‐epiligrin cicatricial pemphigoid; Autoantibodies - immunology; Biopsy, Needle; Cell Adhesion Molecules - analysis; Cell Adhesion Molecules - immunology; Drug Therapy, Combination; Fatal Outcome; Humans; Immunohistochemistry; internal malignancies; Kalinin; laryngeal stenosis; Laryngoscopy - methods; Laryngostenosis - complications; Laryngostenosis - diagnosis; Laryngostenosis - surgery; Lung Neoplasms - complications; Lung Neoplasms - pathology; Lung Neoplasms - therapy; Male; Pemphigoid, Benign Mucous Membrane - complications; Pemphigoid, Benign Mucous Membrane - drug therapy; Pemphigoid, Benign Mucous Membrane - immunology; Pemphigoid, Benign Mucous Membrane - pathology; Risk Assessment; Severity of Illness Index; Tracheostomy - methods
• ISSN: 0385-2407; 1346-8138
• DOI: 10.1111/j.1346-8138.2004.tb00497.x

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.