This is a relatively common disorder, predominantly affecting young men. It is a solitary, unilateral lesion which usually presents in early childhood or adolescence.
Characteristically, there is an irregular brown macular pigmentation on the shoulders, anterior chest or scapular; less commonly, on the forearm, wrist, face or lower limbs.
Hypertrichosis is often seen 1-2 years after puberty. The coarse dark hairs always develop after the pigmentation but may extend to the adjacent normal skin. The condition is often precipitated by sunlight.
The condition is difficult to treat. Electrolysis may be effective in treating the hypertrichosis.
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