Background: DowlingDegos Disease (DDD) is a rare genetic disease of the skin having an autosomal dominant inheritance. It is grouped under reticulated pigmented anomalies and manifests as symmetric, progressive, pigmented macules over flexures, scattered comedolike lesions and pitted acneiform scars. Histopathology is required for definitive diagnosis and to distinguish it from other reticulate pigmentary genodermatoses. Aims: 1. To show the existence of this rare disease in the local population. (2) To stress on the clinicopathological correlation for the definitive diagnosis of the disease. Materials & Methods: Punch biopsies of skin received by the Department of Pathology over a span of two years with Dowling Degos Disease as one of the differential diagnosis, were included in the study. The biopsies were processed as per standard protocol. Diagnosis was confirmed by the histopathological examination. Result: A total of six cases were included in the study, out of which four were histologically confirmed as DDD and one case had features overlapping with Reticulate Acropigmentation of Kitamura (RAPK). The study showed female preponderance. Four cases had family history. The clinical and histopathological findings of each case are discussed. Conclusion: Dowling Degos Disease may not be a rare entity. It should be considered as one of the differential diagnoses for pigmentary disorders. With combined clinical and histopathological evaluation, more cases may come into light in the future and DDD may not remain as a rare disorder.

Keywords: DowlingDegos Disease; Genodermatoses; Reticulate Pigmented Disorders.