Complete Information on Dermatopathia pigmentosa reticularis with Treatment and Prevention

Dermatopathia pigmentosa reticularis (DPR) is a rare genetic condition where the individual is born without fingerprints. It is comparable to Naegeli syndrome, both of which are caused by a specific defect in the keratin 14 protein. Although the gene responsible for dermatopathia pigmentosa reticularis has been identified, there is no cure for this disorder. Those who suffer from DPR should be very mindful of potential occupational hazards. The reticulate pigmentation occurs at birth or during early childhood. Many other dermatologic findings have been associated with this triad. These findings include adermatoglyphia, hypohidrosis or hyperhidrosis, palmoplantar hyperkeratosis, and acral dorsal nonscarring blisters.

The main symptom of dermatopathia pigmentosa reticularis is an individual touching surfaces and not leaving a fingerprint behind. Symptoms include lack of sweat glands, thin hair, brittle nails, mottled skin, and lack of fingerprints. There are virtually no trace of the complex lines and swirls that create fingerprints and toeprints. Other effects of this condition are the inability to sweat, brittle teeth, ridged nails, and dry skin. The loss of fingerprints would never have been considered a disability until today. The real threat to DPR sufferers is the inability to sweat. The hyperpigmentation persists throughout life, showing no tendency of spontaneous fading. The reticulate network of hyperpigmented macules occurs particularly on the trunk, neck, and proximal areas of the limbs.

The patients recommended sun exhibition and less important avoiding traumatism, which can bring about blister shaping. No specific treatment exists for this disorder, except for symptomatic management of some of the associated conditions, such as palmoplantar hyperkeratosis. Hypohidrosis, the main problem for the patients, remains constant. Teeth are always severely affected, leading to early total loss. For hyperkeratosis, topical retinoic acids and keratolytics may be beneficial. No serious complications are known. Some conditions mentioned in the literature are probably coincidental rather than complications. Nonscarring blisters are generally transient and self-healing. Cold compress may suffice.