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Year : 2000  |  Volume : 66  |  Issue : 3  |  Page : 158-159

Dyschromatosis Universalis Hereditaria

Ranju Rai, Inderjeet Kaur, Sanjeev Handa, Bh Kumar 
 

Correspondence Address:
Ranju Rai


Abstract

Dyschromatosis universalis hereditaria is a common disorder in Japan. We report a case in an Indian woman with no family history of the disorder.



How to cite this article:
Rai R, Kaur I, Handa S, Kumar B h. Dyschromatosis Universalis Hereditaria.Indian J Dermatol Venereol Leprol 2000;66:158-159


How to cite this URL:
Rai R, Kaur I, Handa S, Kumar B h. Dyschromatosis Universalis Hereditaria. Indian J Dermatol Venereol Leprol [serial online] 2000 [cited 2019 Aug 21 ];66:158-159
Available from: http://www.ijdvl.com/text.asp?2000/66/3/158/4908


Full Text

 Introduction



Dyschromatoses are a group of disorders characterised by the presence of both hyperpigmented and hypopigmented macules many of which are small in size and irregular in shape. 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). Dyschromatosis symmetrica hereditaria (DSH) was first reported as a clinical entity by Toyama in 1929. [1] It is characterised by a symmetrical distribution of hyperpigmented and hypopigmented macules on the extremities especially over dorsa of the hands and feet.

In 1933 Ichikawa and Hiraga described DUH which was essentially the same disorder but occurring in a generalised as opposed to acral distribution. It has been suggested that DSH might be a s ubtype of DUH but we have to wait for the cloning of the causal genes of these diseases before coming to any definite conclusions. [2]

 Case Report



A 22-year-old unmarried woman with brown hair and black eyes presented to us with chief complaints of multiple hyperpigmented and hypopigmented lesions over arms, legs and buttocks for the last three years. The lesions had started over both the feet and gradually spread upwards towards the thighs over 3 years. Few lesions had appeared during this time over the hands subsequently spreading upwards to the elbows. A few scattered lesions were present on the buttocks. There was no history of photosensitivity. There was no history of handling any chemical directly or any significant history of drug intake. Her palms, soles and mucous membranes were within normal limits. Systemic examination did not reveal any abnormality. There was no history of consanguinity among the parents and none of the family members were affected.

A skin biopsy was taken from both the hyperpigmented and hypopigmented lesions. Epidermis in both the biopsies was thinned out. Basal layer in the 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 lymphomononuclear infitrate.

 Discussion



Dyschromatosis universalis hereditaria is a rare genodermatosis which has been reported most often from Japan. Although majority of cases show autosomal dominant pattern of inheritance, a few have inherited it in an autosomal recessive fashion. In the case of our patient we could not elicit any family history.

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 noticed her present lesions a the age of 19 years. Ephelide - like lesions were seen on the face as have been described earlier. However, our patient had involvement of palms, soles and mucous membranes unlike few patients reported earlier.

Lesions of dyschromatosis symmetrica hereditaria (DSH) have to be differentiated from xeroderma pigmentosum since in both the disorders patients clinically show lesions in the photoexposed areas. However, in dyschromatosis universalis hereditaria lesions occur in the unexposed sites as well. Moreover, the lesions show no atrophy or telangiectasia. The lesions also run a benign course.

It has been suggested in the past that DUH is a disorder of melanocyte number. Based on a recent electron microscopic study it has been suggested that DUH may be a disorder of melanosome production in epidermal melanin units rather than a disorder of melanocyte number. [3] Once thought to be a disease confined to Japanese, DUH is being reported with increasing frequency in other races as well. Findlay and Whiting [4] have described the disorder in two Bantu females and Rycroft et all in an Iraqi girl. From India there have been only four isolated case reports in the past. [6],[7],[8] Despite its rarity it assumes significance as it forms an important differential diagnosis of xeroderma pigmentosum.

References

1Toyama J. Dyschromatosis symmetrica hereditaria. Jap J Dermatol 1929; 29: 95-96.
2Urabe K, Hori Y. Dyschromatosis. Sem Cut Med Surg 1997; 16 No. 1.
3Kim NS, Im S, Kim SC. Dyschromatosis universalis hereditaria. J Dermatol 1997;24:161-164.
4Findlay GH, Whiting DA. Universal dyschromatosis. Br J Dermatol 1971; 85: 66-70.
5Rycroft RJ, Calnan CD, Wells RS. Universal dyschromatosis with small stature and high tone deafness. Clin Exp Dermatol 1977; 2: 45-48.
6Sait MA, Garg BR. Dyschromatosis universalis hereditaria. Indian 3 Dermatol 1985; 51 : 277.
7Pavithran K. Dyschromatosis universalis hereditaria with epilepsy. Indian J Dermatol 1991; 57: 102.
8Gharpuray MB, Tolat SN, Patwardhan SP. Dyschromatosis : its occurrence in two Indian families with unusual features (Letter). Int J Dermatol 1994; 33 : 391 - 392.

 

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