We read with interest the paper by Oley et al. [19921: "Basal cell carcinomas, coarse sparse hair and milia."
The patients described had a variable combination of basal cell carcinomas or nevi, milia, hypotrichosis, coarse sparse hair with shaft dystrophy, facial hyperpigmentation, dry skin and excessive sweating with either autosomal or X-linked dominant inheritance.
We do not think the cases represent a new syndrome, but rather the Bazex-DuprC-Christol syndrome. This rare genodermatosis was first reported as a combination of follicular atrophoderma (85%), basal cell proliferations (14% basal cell nevi, and 28% basal cell carcinomas), hypotrichosis (85%), with either thin or coarse hair, and hypohidrosis (27%) [Bazex et al., 19661. Subsequently, additional findings were reported [Pierard et al., 19711: milia (66%), hair shaft dystrophy with twisted, angulated, and flattened hair (30%) and pigmentation of face and neck. Such manifestations had already been noted by Bazex et al. [19661 in their analysis of previous analogous descriptions. Rates of individual clinical manifestations were studied by Colomb et al. [19891 based on 44 reported cases.
Presence or absence of follicular atrophoderma is not mentioned by Oley et al. [19921. This manifestation may be undiagnosed, even by experienced dermatologists, if not carefully sought [Colomb et al., 19891. Microscopy of the hair shown in the paper is also consistent with the "pseudo pili tortiltrichorrhexis nodosa" hair shaft dystrophy found in the Bazex-Dupr6-Christol syndrome. Moreover, the putative X-linked dominant mode of inheritance reported here was also suggested in most previous reports [PiCrard et al., 1971;Viksnins and Berlin, 1977; Gould and Barker, 19811. Since this syndrome may be heterogenous, we need linkage analyses and heterogeneity tests for further conclusions.