Dermatopathia pigmentosa reticularis: A rare reticulate pigmentary disorder
We report a case of a 21-year old woman who presented with onset of darkening of skin since 6 months of age, which increased progressively to whole of the body within a span of 2-3 years. There was also progressive thickening of skin of palms and soles. Diffuse thinning of hair on scalp was present []. She had never had a hair-cut and hair length was never more than up to lower end of neck. Pubic and axillary hair were also poorly developed. No history of photophobia or any other eye complaints was there. Hearing and sweating were normal. There was no history of similar problem in the family. She was born to a second gravida by normal vaginal delivery. She had 2 siblings who were normal. The morphology of the hair shaft was normal on clinical and microscopic examination. Generalized reticulate hyperpigmentation was present []. Scalp hair were short and there was diffuse thinning []. Nails were dystrophic []. Hyperkeratosis of palms and soles was there []. Poorly developed dermatoglyphics were there []. Oral mucosa and teeth were normal. No ocular or auditory involvement was seen. Her intelligence quotient and stature was estimated to be in the normal range. There was no delay in secondary sexual character development. Routine investigations in the form of complete hemogram, liver function test, renal function test, and chest radiography were normal. Histopathology revealed basal layer degeneration and absence of skin adenexa and melanophages in dermis [] and interface dermatitis.\nA diagnosis of (DPR) with a differential of Naegeli-Franceschetti-Jadassohn (NFJS) was done. Absence of enamel defects and the presence of diffuse non-scarring alopecia favour DPR.\nShe was prescribed keratolytics topically for hyperkeratosis.