RETICULATE HYPERPIGMENTATION

Disorders that ore characterized by a reticulate pattern of pigmentati on are reviewed. Dyskercatosis congenita (DKC) is the prototype of the se. In addition to reticulate hyperpigmentation, mucosal leukoplakia, bone marrow dysfunction, cytogenetic instability, and a predisposition to malignancy are characteristic of DKC. The most common pattern of i nheritance is X-linked, with heterozygous females showing variable exp ression, most likely depending on tissue-specific patterns of random X -inactiivation. Other reticulate pigmentary disorders reviewed include the Naegeli-France-eschetti-Jadassohn syndrome, X-linked reticulate p igmentary disorder, dermatopathia pigmentosa reticularis, Dowling-Dego s disease, dyschromatosis, confluent and reticulated papillomatosis of Gougerot and Carteaud, reticulate acropigmentation of Kitamuro, and R evescz syndrome. Diagnosis, treatment, and sometimes genetic counselin g remain problematic for these entities. The pathophysiology of these disorders is unknown, but will certainly be aided greatly by the futur e identification of the underlying genes. Copyright (C) 1997 by W.B. S aunders Company.