The basal cell naevus syndrome. Report of a family with anosmia and a case of hypogonadotrophic hypopituitarism

A family with the naevoid basal cell carcinoma syndrome is described. Three affected members in one sibship suffered from anosmia. One member has shown a most severe constellation of defects including cataracts, hypertelorism, and anosmia, together with hypogonadism secondary to partial pituitary dysfunction. It is suggested that this latter also represents a previously unreported manifestation of the syndrome.