Dermatopathia pigmentosa reticularis (DPR) is a rare, autosomal dominant congenital disorder that is a form of ectodermal dysplasia. Dermatopathia pigmentosa reticularis is composed of the triad of generalized reticulate hyperpigmentation, noncicatricial alopecia, and onychodystrophy. : 856  DPR is a non life-threatening disease that largely affects the skin, hair, and nails. It has also been identified as a keratin disorder. Historically, as of 1992, only 10 cases had been described in world literature; however, due to recent advances in genetic analysis, five additional families studied in 2006 have been added to the short list of confirmed cases. Signs and Symptoms (Presentation)
Symptoms include lack of sweat glands, thin hair, brittle nails, mottled skin, and lack of fingerprints. It is also characterized by a widespread, early-onset reticulate hyperpigmentation. 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.DPR is very similar to the related Naegeli-Franceschetti-Jadassohn syndrome (NFJS). 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. DPR is distinguished from NFJS by the duration of hyperpigmentation and lack of dental abnormalities. Since these differences are very slight and relatively minimal, researchers recommend that NFJS and DPR be considered a single disorder. Molecular Genetics
The disease interval for DPR was found to harbor 230 genes associated with the condition.
See also
Branchial cleft cyst
Thyroglossal cyst
Lachiewicz Sibley syndrome
List of cutaneous conditions

References
Bibliography
Further reading


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