Dermatopathia pigmentosa reticularis (DPR) is a rare, autosomal dominant[2] congenital disorder that is a form of ectodermal dysplasia.
[7] Those affected may also have adermatoglyphia, abnormal epithelial differentiation, palmoplantar hyperkeratosis of the palms and soles, acral dorsal blistering, as well as hypohidrosis or hyperhidrosis.
Both cause an affected person to lack fingerprints, as well as present a lace-like pattern of hyperpigmentation and hyperkeratosis of the palms of the hands and soles of the feet.
[9] Since these differences are very slight and relatively minimal, researchers recommend that NFJS and DPR be considered a single disorder.
[11] This was found when keratin genes KRT14, KRT16, and KRT17 were reassessed in 2006, revealing pathogenic mutations of KRT14 in all patients with NFJS/DPR, displaying a strong correlation and suggesting a potential causation.
[10] Diagnosis of DPR begins with review of a patient's medical history, laboratory results, and clinical examination of symptoms.
Biopsy and histopathological examination can be used to test for the presence of hyperkeratosis, parakeratosis, follicular plugging, and basal cell melanization, all indicators of DPR.