Dyschromatosis universalis hereditaria (DUH) is a rare pigmentary genodermatosis characterized by reticulated hyper- and hypopigmented macules distributed over the trunk and extremities in otherwise healthy patients. DUH presents in a fashion similar to that of a variety of reticulate and pigmentary dermatoses, some of which are associated with precancerous entities and other comorbidities. It is therefore imperative that the clinician recognize and differentiate these disorders so that appropriate screening and counseling can be offered to the patient. We report a case of DUH in a 13-year-old boy presenting with oral leukokeratosis, with a review of the literature exploring the differential diagnoses.
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