The Bazex Syndrome: follicular atrophoderma with multiple basal cell carcinomas, hypotrichosis and hypohidrosis

Abstract

The Bazex syndrome-follicular atrophoderma, basal cell carcinomas, hypotrichosis, hypohidrosis and some minor skin abnormalities-is described in a Finnish family.Local hypohidrosis was confirmed by measuring the water evaporation rate on the forehead skin. In histological samples from the forehead skin no sweat glands were found. The sweat response due to exercise or metacholine stimulation was slight or negligible.Histologically and ultrastructurally, follicular anomalies were a fundamental feature in the various skin lesions.The Bazex syndrome is a dominantly inherited skin disorder with follicular atrophoderma being the most consistent feature. The patients usually exhibit one or several additional features: multiple basal cell carcinomas, hypotrichosis and localized or general hyphohidrosis. The abnormality was first described by Bazex, Dupre & Christol (1966) in a French family and later by several other authors.We report the syndrome in a Finnish family with five affected members. The case histories, the pedigree and this histological findings are presented. The clinical impression of local hypohidrosis was confirmed by measuring the sweat secretion rates of the patients. Case reports Case IA 13 year-old boy had had since birth numerous small, sharp pits on the back of the hands, the upper arms and the knees. At the age of 3 he was treated, as an inpatient, for atopic

The genetics of hair shaft disorders

“…PT has been reported in association with other genetic hair shaft abnormalities 32,98,105,127,[183][184][185][186][187][188][189] (Table IV).…”

Pharmacological Modulation by Cetirizine and Atropine of the Histamine- and Methacholine-Induced Wheals and Flares in Human Skin

“…Excessive turbulence of the air flux in the cli matic chamber can modify readings, we therefore used a small wind screen in order to protect the investigated area. This method has been used for appreciation of methacholine-induced sweat gland stimulation [23]. In this case, the time necessary to reach stable values is much longer, i.e.…”

Basal cell carcinoma in childhood: Case report and literature review

“…Childhood onset of BCC has previously been associated with albinism, 21,27 Bazex syndrome, 21,28 BCC nevus syndrome, 25,26,[29][30][31][32][33]53 nevus sebaceous, 2,25,26,[34][35][36][37][38][39][40] solid organ transplants, 47,48 xeroderma pigmentosum, 25,29,32,[49][50][51][52] and radiotherapy-treated cancers, such as astrocytoma, 44 ependymoma, 41 hemangioma, 26 Hodgkin lymphoma, 38,46 leukemia, 38,[42][43][44][45][46] medulloblastoma, 22,29,33,46 neuroblastoma, 38 optic nerve glioma, 41 and unspecified malignant tumor. 32 Children are defined to be 18 y...…”