Male-pattern baldness in men with X-linked recessive ichthyosis

Background: X-linked recessive ichthyosis (XRI) is a genetic disorder of ke ratinization with extracutaneous manifestations due to deficiency of steroi d sulfatase (STS). Because STS plays an important role in androgen metaboli sm, and elevated levels of dehydroepiandrosterone sulfate have been reporte d in young men with andogenetic alopecia (AGA), the hypothesis was advanced that men with XRI do not show AGA or develop only mild forms of common bal dness. Methods: Patients with a diagnosis of XRI confirmed by analysis of t he microsomal sulfatases in our clinic between 1984 and 1998, and in whom s tudy of the case histories depicted the typical clinical presentation of XR I, were sent a questionnaire with the Hamilton-Norwood scale of patterns of hair loss in men, inquiring them to designate the condition of their scalp hair, Results and Conclusions: Of 15 questionnaires returned, 7 indicated Hamilton-Norwood 1, 3 Hamirlon-Norwood II, 3 Hamilton-Norwood III; vertex, 1 Hamilton-Nomood IV and 1 Hamilton-Norwood VII, The results of this survey do not support the hypothesis that XRI and AGA are mutually exclusive, in as much as advanced AGA was found among these men. Copyright (C) 2000 S. Ka rger AG, Basel.