Dermatopathia pigmentosa reticularis: A report of a case with delayed onset alopecia and onychodystrophy

Abstract

Dermatopathia pigmentosa reticularis (DPR) is an extremely rare autosomal dominant ectodermal dysplasia that occurs because of mutations in KRT14. It mainly affects the skin, nails, and hair, with a characteristic diagnostic triad of widespread reticulate hyperpigmentation that begins at birth or during early childhood, noncicatricial alopecia (usually mild), and onychodystrophy. Patients with this syndrome may also have adermatoglyphia, palmoplantar hyperkeratosis, hyperhidrosis or hypohidrosis, and acral dorsal nonscarring blisters.1, 2, 3, 4, 5 The first cases of DPR were reported and described in 1958 by Hauss and Oberste-Lehn.1, 6 To date, <20 cases of this syndrome have been reported worldwide.2, 6, 7 We report a case of DPR, and to the best of our knowledge this is the first case of this extremely rare syndrome reported in Iraq. We are urged to report this case because of several factors: a rarity of reports in the literature, an uncanny similarity to other syndromes—in particular, Naegeli-Franceschetti-Jadassohn syndrome (NFJS) and dyskeratosis congenita (DKC)—and the unusual age of presentation of certain diagnostic clinical features in this case.