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.
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.
Matsushima
Satoko
S
Departments of Dermatology, Osaka Red Cross Hospital, Ten'noji-ku, Osaka, Japan.
Horiguchi
Yuji
Y
Honda
Tetsuya
T
Fujii
Shinobu
S
Okano
Takayuki
T
Tanabe
Masahiro
M
Wakayama
Toshiaki
T
Hashimoto
Takashi
T
Yancey
Kim B
KB
D000368:Aged; D001323:Autoantibodies / Q000276:immunology*; D001707:Biopsy, Needle; D015815:Cell Adhesion Molecules / Q000032:analysis / Q000276:immunology*; D004359:Drug Therapy, Combination; D017809:Fatal Outcome; D006801:Humans; D007150:Immunohistochemistry; D007828:Laryngoscopy / Q000379:methods; D007829:Laryngostenosis / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D008175:Lung Neoplasms / Q000150:complications* / Q000473:pathology / Q000628:therapy; D008297:Male; D010390:Pemphigoid, Benign Mucous Membrane / Q000150:complications / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology*; D018570:Risk Assessment; D012720:Severity of Illness Index; D014139:Tracheostomy / Q000379:methods; D000097794:Kalinin
D001323:Autoantibodies; D015815:Cell Adhesion Molecules; D000097794:Kalinin