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October 1989

Universal Inherited Melanodyschromatosis: A Case of Melanosis Universalis Hereditaria?

Arch Dermatol. 1989;125(10):1442-1443. doi:10.1001/archderm.1989.01670220140034

To the Editor.—  In the very rare disease melanosis universalis hereditaria (MUH), a diffuse negroid hyperpigmentation occurs during the first month of life, and melanosomes have a negroid distribution within the keratinocytes. We describe a patient with MUH who was distinctive in that he had extensive mottling and maintained a caucasoid pattern of distribution of melanosomes.

Report of a Case.—  A 37-year-old man, referred to us because of lichen simplex, exhibited a diffuse hyperpigmentation that was particularly intense on his face and neck. On a brownish background, many darker spots stood forth on his trunk (Fig 1), palms, and soles. Some pigmented areas were also present on the oral mucous membrane. The genitalia were completely dark brown. On the trunk, a few 0.5-mm-wide hypopigmented macules were also present. The hair was black and fairly straight; the eyes were dark brown.Reportedly, the pigmentation began 1 to 2 months after birth,

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