Dominant X-linked genodermatosis due to a mutation of the UBE2A gene (Xq24 - q27.1). It predisposes to early occurrence of basal cell carcinomas. Onset in the neonatal period or in early childhood. Follicular atrophy of the dermis (face, back of hands and feet, extension surfaces of knees and elbows), hypotrichosis (scalp, eyebrows), hypohidrosis, many milium grains on the face, occurrence of basal cell carcinomas (face) in 40% of patients, in the second or third decade.

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Updated: November 2019