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Department of Dermatology, Wuxi No.2 People's Hospital, Wuxi 214002, Jiangsu, China

Reticular pigmented anomaly of the flexures(RPAF)is a rare autosomal dominant genodermatosis characterized by disfiguring reticulate hyperpigmentation of the flexures. The pigmentation of RPAF is often symmetrical, extensive, asymptomatic and progressive with age. With more and more RPAF pedigrees reported, its clinical manifestation and pathogenesis have become a hotspot of recent researches. This review focuses on the pathogenisis, clinical and histopathological manifestations, differential diagnosis, and treatment of this disease.