Two patients with hereditary hypotrichosis congenita are reported. The family was affected in five generations. Our patients belong to the same family Marie Unna originally investigated. The histological findings correspond with the clinical picture. In the second case, we suspect an early latent stage of this hair abnormity on account of the condition of the hair as well as our microscopical findings. According to literature, seven families suffering from this disease are known up to now.

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