[Haber's syndrome. First French family (2 cases)].
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).
Binet
O
O
Audefray
D
D
Beltzer-Garelly
E
E
Gauchy
O
O
Cesarini
J P
JP
D000328:Adult; D005148:Facial Dermatoses / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D005260:Female; D005602:France; D006801:Humans; D007642:Keratosis / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010859:Pigmentation Disorders / Q000175:diagnosis; D013577:Syndrome; D014860:Warts / Q000175:diagnosis / Q000235:genetics
Le syndrome de Haber. Première famille française (deux cas).