DERMATOPATHIA PIGMENTOSA RETICULARIS
By Gary M. White, MD
Dermatopathia pigmentosa reticularis (DPR) is a very rare autosomal dominant ectodermal dysplasia characterized by generalized reticulate hyperpigmentation, nonscarring alopecia, and onychodystrophy.
- DPR is caused by mutations in the KRT14 gene.
- Additional features can include the absence of or decreases in adermatoglyphia, hypohidrosis or hyperhidrosis, palmoplantar hyperkeratosis, and acral nonscarring blisters.
Fourteen and 11-year-old siblings presented with generalized reticulate pigmentation since birth as well as generalized sweating and recurrent blisters, mainly over the soles. Furthermore, they reported progressive hair thinning and abnormal nail growth. Indian J Dermatol 2016;61:468
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