Two Cases of Anti-Epiligrin Cicatricial Pemphigoid With and Without Associated Malignancy
Satoshi Fukushima, Kiyofumi Egawa, Hazuki Nishi, Shoji Wakasugi, Norito Ishii, Takashi Hashimoto, Kim B. Yancey, Hironobu Ihn
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, in¬direct immunofluorescence demonstrated circu¬lating 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 β3 subunit of laminin 5 and IgG antibodies of patient 2 reacted with the 165-kDa and 145-kDa α3 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.