Dermatopathia Pigmentosa Reticularis: Report of New Cases and Literature Review

Dermatopathia pigmentosa reticularis (DPR) was a rare autosomal dominant ectodermal dysplasia caused by mutations in the KRT14 gene, characterized by a triad of generalized reticulate hyperpigmentation, nonscarring alopecia, and onychodystrophy. The study reported on two siblings from Saudi Arabia, marking the first documented cases in the region. The siblings exhibited symptoms from birth, including reticulate pigmentation, sweating, recurrent blisters, hair thinning, and nail abnormalities. Histopathological examination showed typical features of DPR, and electron microscopy revealed normal hair shaft structures. DPR was distinguished from Naegeli–Franceschetti–Jadassohn syndrome by the persistence of skin hyperpigmentation and absence of dental anomalies.