Two cases of anti-epiligrin cicatricial pemphigoid with and without associated malignancy.
Anti-epiligrin cicatricial pemphigoid (AECP) is a chronic, mucous membrane-dominated, subepithelial blistering disease characterized by circulating anti-basement membrane zone auto-antibodies to laminin 5. Recent studies have shown that people with AECP have an increased relative risk for malignant tumours. In this report we describe two patients with AECP. In both cases, indirect immunofluorescence demonstrated circulating IgG anti-basement membrane auto-antibodies that bound to the dermal side of 1M NaCl split normal skin. Immunoblotting using laminin 5 purified from keratinocyte extract as a substrate showed that the IgG antibodies of patient 1 reacted with the 140-kDa beta3 subunit of laminin 5 and IgG antibodies of patient 2 reacted with the 165-kDa and 145-kDa alpha3 subunits. Patient 1 had prostate carcinoma and his blistering was resistant to therapy. Patient 2 had no detectable malignancy and treatment with doxycycline was successful.
Fukushima
Satoshi
S
Department of Dermatology & Plastic and Reconstructive Surgery, Graduate School of Medical and Pharmaceutical Sciences, Kumamoto University, Kumamoto, Japan. 056r5162@med.stud.kumamoto-u.ac.jp
Egawa
Kiyofumi
K
Nishi
Hazuki
H
Wakasugi
Shoji
S
Ishii
Norito
N
Hashimoto
Takashi
T
Yancey
Kim B
KB
Ihn
Hironobu
H
D000230:Adenocarcinoma / Q000150:complications; D000368:Aged; D000369:Aged, 80 and over; D000890:Anti-Infective Agents / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood; D015815:Cell Adhesion Molecules / Q000276:immunology*; D003622:Dapsone / Q000627:therapeutic use; D004318:Doxycycline / Q000627:therapeutic use; D004351:Drug Resistance; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D008297:Male; D010390:Pemphigoid, Benign Mucous Membrane / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D011471:Prostatic Neoplasms / Q000150:complications*; D000097794:Kalinin
D000890:Anti-Infective Agents; D001323:Autoantibodies; D015815:Cell Adhesion Molecules; D003622:Dapsone; D004318:Doxycycline; D007074:Immunoglobulin G; D000097794:Kalinin