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December 1990

Universal Dyschromatosis Associated With Photosensitivity and Neurosensory Hearing Defect

Author Affiliations

Department of Dermatology Tokyo Teishin Hospital Fujimi 2-14-23 Chiyoda-ku Tokyo 102, Japan

Arch Dermatol. 1990;126(12):1659-1660. doi:10.1001/archderm.1990.01670360127036

To the Editor.—  Dyschromatosis universalis hereditaria (DUH) is a congenital dyschromatosis that usually shows autosomal dominant inheritance.1 Dyschromatosis universalis hereditaria shows diffuse mottled hyperpigmentation and hypopigmentation in otherwise healthy patients.1-4 We observed a middle-aged woman whose clinical features were similar to DUH, but whose histologic picture showed abnormally melanin-laden melanocytes in the basal cell layer. Moreover, she was photosensitive to both ultraviolet (UV) B and UVA and had a mild neurosensory hearing defect.

Report of a Case.—  A 37-year-old woman with multiple mottled hyperpigmented and hypopigmented macules (0.2 to 0.5 cm in diameter) over her entire body, except for her palms and soles, was seen. There was no reticulate pattern of pigmentation (Fig 1). She had had these macules since childhood. She stated that no pigmentary disorder has been found in her relatives. She experienced severe sunburn at 3 months of age and every summer thereafter. Phototesting revealed

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