The Network of Mystery: Unraveling Atypical Dermatopathia Pigmentosa Reticularis

Dermatopathia pigmentosa reticularis (DPR) is a rare ectodermal dysplasia characterized by generalized reticulate hyperpigmentation, nonscarring alopecia, and onychodystrophy, caused by autosomal dominant mutations in KRT14. Fewer than 25 cases have been reported worldwide. We present a 27-year-old male with widespread hyperpigmentation since childhood, starting on the trunk and spreading to the extremities, face, and oral mucosa, sparing the palms and soles. He also had punctate palmoplantar keratoderma and absent fingerprints. Histopathology showed thinning of the epidermis, basal layer hyperpigmentation, and pigmentary incontinence. DPR was diagnosed based on clinical and histological findings, with Naegeli-Franceschetti-Jadassohn syndrome (NFJS) considered less likely. Unlike NFJS, the patient had no alopecia, dental, or corneal abnormalities. DPR has no specific treatment, and management focuses on symptomatic relief. This case contributes to the limited literature on DPR, highlighting the importance of distinguishing it from similar disorders.