SASH1-associated skin dyschromia includes classic DUH and the lentiginous phenotype; it predominantly involves sun-­exposed areas with or without dyschromatosis or concomitant palmoplantar keratoderma and skin carcinoma.20 One patient with DUH and a SASH1 mutation (c.1529G > A) presented with lentigines on the face and hands, which gradually spread to the trunk and extremities, without the involvement of the palms, soles, and mucosa. Here, SASH1 is linked to Palmoplantar keratoderma.