dyschromatoses are a group of disorders characterized by the presence of small irregular hyper- and hypopigmented macules . it is a spectrum of diseases which includes dyschromatosis universalis hereditaria ( duh ) , dyschromatosis symmetrica hereditaria ( dsh ) , or acropigmentation of dohi and a segmental form called unilateral dermatomal pigmentary dermatosis ( udpd ) . it is characterized by a symmetrical distribution of hyper- and hypopigmented macules on the extremities , especially over dorsa of the hands and feet . in 1933 ichikawa and higara described duh which was essentially the same disorder , but occurring in a generalized as opposed to acral distribution . 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 . there was no history of handling any chemical directly or any significant history of drug intake . physical examination revealed numerous asymptomatic , generalized , 0.5 - 1 cm hyperpigmented macules interspersed with spotty hypopigmented macules . there was no apparent atrophy , erythema , or telangiectasia [ figures 1 and 2 ] . 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 . the lesions were dense on the limbs and trunk with sparing of the palms and soles multiple dense hyper - and hypopigmented lesions over arms a skin biopsy was taken from both the hyper- and hypopigmented lesions . 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 3 and 4 ] . basal layers showed mild vacuolar change , papillary dermis with melanin incontinence , and dilated dermal vessels ( hematoxylin and eosin ( h and e , 10 ) histopathology of the skin lesion : in high power , section shows increase in pigment extending into the stratum spinosum . dermis showed mild perivascular and periadnexeal lymphomononuclear infitrate ( h and e ,  10 ) the 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 . although majority of cases show autosomal dominant pattern of inheritance , a few have inherited it in an autosomal recessive fashion . a variable autosomal mode of inheritance of duh has also been described . in our case , two major types have been described , based on distribution of lesions as follows : duh , first reported in 1933 by ichikawa and higara , in which mixtures of hyper- and hypopigmented macules occur all over the body ; and dsh , also known as acropigmentation of dohi , described in 1929 by toyama , which is a localized form involving only the acral areas . however , a few cases of duh were described among europeans , south americans , indians , and saudi arabians and in tunisia . in duh , facial lesions were seen in almost 50% of affected individuals , but involvement of palms and soles is unusual . the histopathology typically shows a focal increase or decrease in melanin content of the basal layer ( depending on the type of the lesion biopsied ) and occasionally pigmentary incontinence . in a recent ultrastructural skin investigation , nuber et al . indicated that duh is a disorder of melanosome synthesis rate or in melanocyte activity and not a disorder of melanocyte number . of the 37 previously reported cases from japan , 15 were men and 22 were women . eighty - two percent of patients had clinical symptoms before the age of 6 years . our patient gave a history that her lesions presented at the age of 3 years . duh may be associated with abnormalities of dermal connective tissue , nerve tissue , or be associated with other systemic complications . lesions of dsh have to be differentiated from xeroderma pigmentosum , since in both the disorders patients clinically show lesions in the photoexposed areas . . the lesions also run a benign course . generally , duh does not progress or worsen with age . in all reports other differential diagnosis of duh to be considered are dsh , dyskeratosis congenital , generalized dowlinge - degos disease , incontinentia pigmenti ( bloch - sulzberger ) , naegelie - franceschetti - jadassohn syndrome , chronic arsenic toxicity , and chronic radiodermatitis . once thought to be a disease confined to japanese , duh is being reported with increasing frequency in other races as well . from india these patients often suffer from depression because of cosmetic disfigurement so simultaneous counseling and psychiatric consultation can help them lead more fulfilling and productive lives . role of nbuvb as a treatment modality should be evaluated and tried for better cosmetic appearance .
dyschromatosis universalis hereditaria ( duh ) is a rare genodermatosis reported initially and mainly in japan . 
 however , subsequent cases have been reported from other countries . 
 we report a case of duh in a south indian woman with a positive family history with cosmetic disfigurement and severe psychological impairment .