Male-pattern baldness is common in men with X-linked recessive ichthyosis.
BACKGROUND
X-linked recessive ichthyosis (XRI) is a relatively common genetic disorder of keratinization caused by deficiency in steroid sulfatase (STS) activity. STS appears to play an important role in testosterone metabolism. Therefore it has been discussed that the presence of normally functioning STS may be a presupposition for the development of androgenetic alopecia (AGA).


METHODS
Patients with the diagnosis of XRI were sent questionnaires.


CONCLUSIONS
We reviewed 26 cases with XRI and noticed 11 patients with AGA in an advanced stage. The existence of two pathways for the steroid biosynthesis may be the explanation for a compensatory mechanism in XRI males. The Delta5 pathway depends on steroid sulfate activity, whereas the working Delta4 pathway produces AGA in XRI males.
Axt-Gadermann
M
M
TOMESA Clinic, Bad Salzschlirf, Germany.
Schlichting
M
M
Küster
W
W
D000328:Adult; D017677:Age Distribution; D000368:Aged; D000505:Alopecia / Q000175:diagnosis* / Q000453:epidemiology*; D015897:Comorbidity; D006801:Humans; D016114:Ichthyosis, X-Linked / Q000175:diagnosis* / Q000453:epidemiology*; D015994:Incidence; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D018570:Risk Assessment; D012494:Sampling Studies; D011795:Surveys and Questionnaires