Dermatopathia Pigmentosa Reticularis: A Case Report with Delayed Onset Alopecia and Onychodystrophy

The document reported a case of a 24-year-old Iraqi man with dermatopathia pigmentosa reticularis (DPR), a rare autosomal dominant ectodermal dysplasia caused by mutations in KRT14. The patient exhibited the diagnostic triad of DPR: widespread reticulate hyperpigmentation, noncicatricial alopecia, and onychodystrophy. Notably, the alopecia and nail abnormalities appeared later in life, at ages 19 and 23, respectively. The case highlighted the importance of considering DPR in patients with early-onset reticulate hyperpigmentation, even if other symptoms manifest later. The patient also had adermatoglyphics, sweating abnormalities, and heat intolerance, which are associated with DPR. Elevated serum cortisol and hepatomegaly were observed, though their relevance to DPR was uncertain.