Dermatology Department, Habib Thameur Hospital-Tunis.
Int J Dermatol. 2010 Apr;49(4):396-401. doi: 10.1111/j.1365-4632.2010.04403.x.
Most of the published reports on Hailey-Hailey disease (HHD) come from European and Asian countries. We report here the clinical and genetic investigation of 20 patients affected with HHD in Tunisia.
Affected individuals from three large teaching hospitals in Tunis were recruited for the study over a 25-year period. Nine patients were identified through the active files and examined together with their family members that were visited in their respective regions. We have clinically examined in total 65 individuals and then identified 11 new cases. Patients were included on the basis of evocative skin lesions, biopsy proven HHD and negative immunofluorescence. Investigations to rule out fungal, bacterial and viral infections were done according to clinical symptoms.
Twenty patients (12 males and 8 females) from 8 families were included in the present study with more than 55% that were undiagnosed before this investigation. Four patients had mild disease, eight had moderate disease and another eight had severe disease, among whom seven were females. Parental consanguinity was found in 7 cases out of 20 cases (35%). The neck region was first affected in half (4/8) of the male patients. Groins were first affected in 42% (5/12) of the female patients. Depression complicated the course of the disease in two female patients with severe HHD. We report an original association of supernumerary nipples with HHD in two sisters from the north of Tunisia. In 10 patients, the disease has become less troublesome with aging.
HHD is underestimated. Physicians must be aware of this disease in case of resistant intertriginous dermatosis especially with a positive family history as nine out of 20 patients were misdiagnosed.
大多数关于 Hailey-Hailey 病（HHD）的已发表报告来自欧洲和亚洲国家。我们在此报告 20 例突尼斯 HHD 患者的临床和遗传研究。
在 25 年的时间里，我们从突尼斯的三家大型教学医院招募了受影响的个体进行研究。通过主动档案确定了 9 名患者，并与在各自地区就诊的家庭成员一起进行了检查。我们总共检查了 65 个人，然后确定了 11 例新病例。根据活检证实的 HHD 和阴性免疫荧光，在出现提示性皮损的基础上确定了患者。根据临床症状进行了排除真菌、细菌和病毒感染的检查。
本研究共纳入 8 个家庭的 20 名患者（12 名男性和 8 名女性），其中超过 55%的患者在本次调查之前未被诊断。4 名患者病情较轻，8 名患者病情中度，另 8 名患者病情严重，其中 7 名是女性。20 例中有 7 例（35%）存在父母近亲结婚。颈部是一半（4/8）男性患者的首发部位。腹股沟是 42%（5/12）女性患者的首发部位。2 例病情严重的女性患者出现了抑郁，使病情复杂化。我们报告了突尼斯北部的 2 位姐妹与 HHD 相关的一个新的附加乳头。在 10 名患者中，随着年龄的增长，疾病的困扰程度降低。
HHD 被低估了。医生必须注意这种疾病，尤其是在有阳性家族史的情况下，因为 20 例患者中有 9 例被误诊。
