Basal cell carcinomas, multiple milia of face and
limbs, increased sweating and facial pigmentation, and sparse scalp and body
Incidence is not known.
Inheritance seems to be either autosomal dominant or X-linked dominant.
Main features are sparse and coarse scalp hair,
sparse body hair, and multiple, often big milia on face and limbs, which
tend to disappear around puberty. Basal cell carcinomas (most often
multiple) develop in early adult life. Some patients complain about
excessive sweating. Some researchers believe it is the same entity as
Atropine and other parasympatholytic
drugs may reduce the excessive sweating, which could otherwise render taping for the
endotracheal tube, the intravenous cannula, and ECG electrodes challenging. However,
temperature must be controlled to prevent hyperthermia.
Bazex Syndrome: X-linked dominant inherited disorder characterized
by follicular atrophoderma in combination with hypotrichosis, hypohidrosis,
and nevoid basal cell carcinoma.
Oley CA, Sharpe H, Chenevix-Trench G: Basal cell carcinomas, coarse
sparse hair, and milia. Am J Med Genet
Vabres P, de Prost Y: Bazex-Dupre-Christol syndrome: A possible diagnosis
for basal cell carcinomas, coarse sparse hair, and milia [letter].
Am J Med Genet