This article mainly reported a rare presentation and histopathologic finding of woolly hair nevus. Woolly hair nevus is a rare disease whose diagnosis is challenging. We present a case of this condition presenting in a 27-year-old healthy male. We describe a histology pattern consisting in the presence of several terminal hair follicles ending in the same dilated follicular infundibulum, a perifollicular lymphocytic infiltrate and an excessive amount of normal apocrine glands in deep reticular dermis, some findings non-previously reported.
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