Male-pattern baldness in men with X-linked recessive ichthyosis.
BACKGROUND
X-linked recessive ichthyosis (XRI) is a genetic disorder of keratinization with extracutaneous manifestations due to deficiency of steroid sulfatase (STS). Because STS plays an important role in androgen metabolism, and elevated levels of dehydroepiandrosterone sulfate have been reported 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 baldness.


METHODS
Patients with a diagnosis of XRI confirmed by analysis of the microsomal sulfatases in our clinic between 1984 and 1998, and in whom study of the case histories depicted the typical clinical presentation of XRI, 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.


CONCLUSIONS
Of 15 questionnaires returned, 7 indicated Hamilton-Norwood I, 3 Hamilton-Norwood II, 3 Hamilton-Norwood III-vertex, 1 Hamilton-Norwood 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.
Trüeb
R M
RM
Department of Dermatology, University Hospital of Zurich, Switzerland. ramitru@derm.unizh.ch
Meyer
J C
JC
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000505:Alopecia / Q000473:pathology*; D019314:Dehydroepiandrosterone Sulfate / Q000378:metabolism; D005808:Genes, Recessive; D006801:Humans; D006923:Hymecromone / Q000031:analogs & derivatives / Q000378:metabolism; D016114:Ichthyosis, X-Linked / Q000235:genetics / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires
C019492:4-methylumbelliferyl sulfate; D006923:Hymecromone; D019314:Dehydroepiandrosterone Sulfate