Dyschromatosis universalis hereditaria
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Synopsis
DUH is characterized by asymptomatic, reticular, hyper- and hypopigmented macules of irregular shape and size that are distributed diffusely over the trunk and extremities. While involvement of the face is present in roughly 50% of affected individuals, palms and soles are typically spared. Dyspigmentation usually appears in infancy or early childhood and persists throughout life.
In general, DUH is skin limited, but there have been reports of associated conditions, including cataracts, diabetes, glaucoma, hypothyroidism, intellectual disability, photosensitivity, primary ovarian insufficiency, sensorineural hearing loss, small stature, tuberous sclerosis, and X-linked ocular albinism.
Related topic: dyschromatosis symmetrica hereditaria
Codes
ICD10CM:Q82.8 – Other specified congenital malformations of skin
SNOMEDCT:
239082002 – Dyschromatosis universalis
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
Congenital and inherited pigmentary disorders:- Reticulate acropigmentation of Kitamura – reticular hyperpigmentation over dorsal hands and feet without hypopigmented macules, atrophic skin, palmoplantar pitting
- Dyschromatosis symmetrica hereditaria – reticular hypo- and hyperpigmented macules limited to face and acral areas
- Xeroderma pigmentosum – photosensitivity with progressive xerosis, atrophy, and telangiectasias on sun-exposed areas, predilection for skin cancers at a young age, autosomal recessive inheritance
- Reticulate pigmented anomaly of flexures (Dowling-Degos disease) – brown-black hyperpigmentation of the flexures, often in axilla, neck, and inframammary areas, perioral acneiform scars
- Dermatopathia pigmentosa reticularis – noncicatricial alopecia, onychodystrophy
- Naegeli-Franceschetti-Jadassohn syndrome – abnormal sweating, adermatoglyphia, and palmoplantar keratoderma
- Dyskeratosis congenita – idiopathic pulmonary fibrosis, nail dystrophy, palmoplantar hyperkeratosis, premalignant leukoplakia, progressive pancytopenia, telangiectasias
- Amyloidosis cutis dyschromica – biopsy showing amyloid deposition
- Vitiligo
- Contact or drug-induced leukoderma
- Idiopathic guttate hypomelanosis
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References
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Last Reviewed:05/15/2018
Last Updated:01/13/2022
Last Updated:01/13/2022