Crotty C P, Scheen S R, Masson J K, Winkelmann R K
Arch Dermatol. 1981 Sep;117(9):540-2.
Familial benign chronic pemphigus (Hailey and Hailey disease) is a rare, recalcitrant, often disabling genodermatosis that may not respond to conservative dermatologic therapy. We describe herein five patients with intertriginous familial benign chronic pemphigus who underwent excision and split-thickness skin grafting. All were men whose duration of disease ranged from one to 38 years. Follow-up evaluations ranging from ten months to nine years revealed no recurrence in graft sites in three patients, mild recurrence in one patient after eight years, and one death from pulmonary embolus in the postoperative period. The occurrence of familial benign chronic pemphigus around graft edges was a universal, but relatively minor, problem. Surgical excision provided definite relief from an otherwise disabling disease in four of our patients and a satisfactory improvement in life-style.
家族性良性慢性天疱疮（黑利-黑利病）是一种罕见的、顽固的、常导致功能障碍的遗传性皮肤病，可能对保守的皮肤科治疗无反应。我们在此描述5例患有间擦疹型家族性良性慢性天疱疮的患者，他们接受了切除和中厚皮片移植手术。所有患者均为男性，病程从1年到38年不等。随访时间从10个月到9年，结果显示3例患者移植部位未复发，1例患者在8年后轻度复发，1例患者在术后因肺栓塞死亡。移植边缘出现家族性良性慢性天疱疮是一个普遍但相对较小的问题。手术切除为我们的4例患者明确缓解了原本导致功能障碍的疾病，并使生活方式得到了令人满意的改善。
