Binet O, Audefray D, Beltzer-Garelly E, Gauchy O, Cesarini J P
Ann Dermatol Venereol. 1986;113(1):43-50.
Haber's syndrome is a genodermatosis first described by Sanderson and Wilson (17) in 1965. Pursuing Dr. Haber's work, these authors reported three cases from one single family presenting with the dermatosis. A second family was reported by Seiji and Otaki (17) in 1971; Izaka described two cases (pedigree unknown, 13) and two more by Kikuchi (10, 13) in 1981 and 1983. We report here two new cases (brother and sister) discovered in 1984 (7) and representing the first French family. The dermatosis is characterized by clinical, genetic and histological criteria. It is transmitted as an autosomal dominant trait (fig. 1 and 2). Clinically, the face is affected by a rosacea-like dermatitis beginning in childhood and proceeding with pustular flare-ups. The facial lesions are frequently aggravated by exposure to the sun. The patients have pigmented keratotic lesions of the trunk resembling seborrhoeic warts. These lesions are very numerous and begin, on average, during the second decade of life. Some patients present with lesions that are diagnosed as Bowen's disease at histological examination (7, 17). Xerosis cutis is also present. Microscopically, the facial lesions consist of a necklace of basaloid cells around the hair and sebaceous follicles. The keratotic lesions have been reported as either intra-epidermal epitheliomas (17), or seborrheic warts without signs of malignancy (7, 10, 13), or equivalents of the facial lesions (19).
哈伯综合征是一种遗传性皮肤病，1965年由桑德森和威尔逊首次描述。继哈伯博士的研究之后，这些作者报告了来自一个家族的三例患有这种皮肤病的病例。1971年，清二和大泷报告了另一个家族；伊坂描述了两例（家系不明），菊池在1981年和1983年又报告了两例。我们在此报告1984年发现的两例新病例（兄妹），这是首个法国家族的病例。这种皮肤病有临床、遗传和组织学标准。它以常染色体显性特征遗传（图1和图2）。临床上，面部会出现类似酒渣鼻的皮炎，始于儿童期，并伴有脓疱发作。面部病变经常因日晒而加重。患者躯干有色素沉着的角化性病变，类似脂溢性疣。这些病变非常多，平均始于生命的第二个十年。一些患者在组织学检查时出现被诊断为鲍恩病的病变。皮肤干燥也存在。显微镜下，面部病变由围绕毛发和皮脂腺毛囊的基底样细胞链组成。角化性病变被报告为表皮内上皮瘤，或无恶性迹象的脂溢性疣，或面部病变的类似物。
