Dyschromatosis universalis hereditaria: Infrequent genodermatoses in India
A 28-year-old unmarried woman from rural south India presented to us with chief complaints of multiple hyper- and hypopigmented lesions over arms, legs, trunk, and buttocks since she was 3-years-old, she was apparently normal prior to that age. The lesions had started over both the legs and gradually spread upwards towards the thighs. Few lesions had appeared during this time over the hands subsequently spreading upwards to the elbows. Later, lesions developed over the buttocks and trunk also. There was no history of photosensitivity or photophobia. There was no history of handling any chemical directly or any significant history of drug intake. Her mental status was subnormal with depression.\nPhysical examination revealed numerous asymptomatic, generalized, 0.5-1 cm hyperpigmented macules interspersed with spotty hypopigmented macules. The lesions were dense on the limbs and trunk. Palms and soles were not involved. Her face showed mild involvement. The hair, nails, teeth, and mucosae appeared normal. There was no apparent atrophy, erythema, or telangiectasia [Figures and ]. Systemic examination did not reveal any abnormality. Other routine investigations and chest X-ray were within normal limits. Venereal disease research laboratory (VDRL) and human immunodeficiency syndrome test was negative. There was no history of consanguinity among the parents. She reported that her father and sister had similar lesions, but in a milder form. She was depressed since she was not married due to the cosmetic disfigurement and felt worthless, we counseled the patient and also referred her for psychiatric consultation.\nA skin biopsy was taken from both the hyper- and hypopigmented lesions. Section studies shows epidermis in both the biopsies was mildly atrophic with hyperkeratosis. Basal layers showed mild vacuolar change, papillary dermis with melanin incontinence, and dilated dermal vessels. Hyperpigmented lesions showed increase in pigment extending into the stratum spinosum; whereas, in the hypopigmented lesions it showed decrease in pigment. Dermis in both the biopsies showed mild perivascular and periadnexeal lymphomononuclear infitrate [Figures and ].\nThe patient is currently being treated with narrowband ultraviolet B (NBUVB) therapy, has completed 20 sessions and feels her lesions have improved. Also, was put on escitalopram 10 mg in the morning and clonazepam 0.5 mg at night for depression. Patient feels after the counseling and with treatment, is happier with the cosmetic appearance of the lesions and has started working too.