Dainichi Teruki, Takeshita Hiromichi, Moroi Yoichi, Urabe Kazunori, Yoshida Mariko, Hisamatsu Yoshiko, Komai Ayako, Duan Hong, Koga Tetsuya, Hashimoto Takashi, Furue Masutaka
Department of Dermatology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Eur J Dermatol. 2005 May-Jun;15(3):189-93.
Cicatricial pemphigoid (CP) is an autoimmune bullous disease accompanied by mucosal lesions. The majority of patients with CP have autoantibodies against BP180. Anti-laminin 5 (epiligrin) CP is relatively rare. It is known that, in most cases, circulating autoantibodies against laminin 5 in these patients recognize the alpha3 and/or beta3 subunits of this molecule. Here we report a case of anti-laminin 5 CP, which showed IgG autoantibodies against the gamma2 subunit of laminin 5 alone. A 50-year-old woman suffered from skin blistering on the trunk and extremities and severe mucosal lesions in the eyes, oral cavities and laryngopharynx. Despite potent systemic steroids, the mucosal lesions and some parts of the skin lesions persisted. Salazosulfapyridine was of value in controlling the laryngopharyngeal lesions and persistent cutaneous blistering, and cyclophosphamide had definite effects especially on ocular lesions. Anti-laminin 5 autoantibodies became undetectable in serum from the patient after the disease was controlled.
瘢痕性类天疱疮（CP）是一种伴有黏膜损害的自身免疫性大疱性疾病。大多数CP患者有抗BP180自身抗体。抗层粘连蛋白5（表皮整联配体蛋白）CP相对少见。已知在大多数情况下，这些患者中循环的抗层粘连蛋白5自身抗体识别该分子的α3和/或β3亚基。在此我们报告一例抗层粘连蛋白5 CP病例，该病例仅显示针对层粘连蛋白5γ2亚基的IgG自身抗体。一名50岁女性，躯干和四肢出现皮肤水疱，眼、口腔和喉咽部有严重黏膜损害。尽管使用了强效全身性类固醇，但黏膜损害和部分皮肤损害仍持续存在。柳氮磺胺吡啶对控制喉咽部损害和持续性皮肤水疱有作用，环磷酰胺尤其对眼部损害有确切疗效。疾病得到控制后，患者血清中抗层粘连蛋白5自身抗体检测不到。
