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Dyschromatosis universalis hereditaria: report of six cases from a family

Published on Jan 1, 2016in Dermatology Online Journal
Swapan Sardar1
Estimated H-index: 1
,
Anupam Das3
Estimated H-index: 3
,
Debabrata Bandyopadhyay9
Estimated H-index: 9
Abstract
Dyschromatosis universalis hereditaria (DUH) is a rare pigmentary disorder characterized by the presence of mottled hyperpigmented and hypopigmented macules over the trunk, extremities, and face. We have presented a case series comprised of six members of a family who had numerous hyperpigmented and hypopigmented macules distributed all over the body. Histological findings were suggestive of dyschromatosis universalis hereditaria.
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References12
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#1Prabhu Namitha (Bangalore Medical College and Research Institute)H-Index: 2
#2Sarvajnamurthy A Sacchidanand (Bangalore Medical College and Research Institute)H-Index: 7
Dyschromatosis is a pigmentary genodermatosis which presents with hyper and hypopigmented skin lesions giving a mottled appearance. It is a rare entity in India reported mainly in the East Asian population. Classically, two forms have been described; dyschromatosis universalis hereditaria (DUH) and dyschromatosis symmetrica hereditaria. Here we report four cases of DUH and one case of dyschromatosis symmetrica hereditaria from India.
2 CitationsSource
Dyschromatosis universalis hereditaria (DUH) is a rare, autosomal dominant genodermatosis with a peculiar reticulate pigmentary change, consisting of hyperpigmented macules mingled with hypopigmented lesions to give an overall impression of mottling. We herein report a case of DUH with adermatoglyphia in a young male with family history of the disorder.
2 CitationsSource
Dyschromatosis universalis hereditaria is a rare genodermatosis characterized by hyper- and hypopigmented macules in a reticulate pattern. Here, we present a case of DUH with involvement of the palms.
2 CitationsSource
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.
2 CitationsSource
Dyschromatosis universalis hereditaria (DUH) is a rare genodermatosis mainly described in asian subjects. Here, we report a case of a caucasian 11-year-old boy with DUH and an unaffected twin brother. Parents were not consanguineous. A review of the main phenotical, clinical and hystological aspects of this rare entity is exhibited. Differential diagnose might be stablished with several pigmentary disorders, so Dermatologist might have this entity in mind to make a correct diagnose, specially in...
5 CitationsSource
4 Citations
#1C. Y. Wu (NDMC: National Defense Medical Center)H-Index: 1
#2W. H. Huang (NDMC: National Defense Medical Center)H-Index: 1
9 CitationsSource
Tuberous sclerosis is an autosomal dominant disease due to mutations in two genetic loci, characterized by hamartoma formation in the skin, nervous system, heart, kidney, and other organs. Dyschromatosis universalis hereditaria is an autosomal dominant genodermatosis, characterized by small hyperpigmented and hypopigmented macules, uniformly distributed over the entire body. The face is rarely involved, and the palms, soles, and mucous membranes are usually spared. We report a case of tuberous s...
6 CitationsSource
#1Iqbal A. Bukhari (King Faisal University)H-Index: 9
Last. M. StuhrmannH-Index: 2
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18 CitationsSource
#1Gang Wang (Fourth Military Medical University)H-Index: 23
#2C. Li (Fourth Military Medical University)H-Index: 9
Last. Yufeng Liu (Fourth Military Medical University)H-Index: 16
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Summary Two cases of dyschromatosis universalis hereditaria (DUH) from a Chinese family are presented. Case 1 was a 62-year-old woman who had a generalized and progressive hyper- and hypopigmentation of the skin from the age of 8 years. Her brother had also developed a similar skin pigmentary defect from about the same age. Histopathological and ultrastructural examination of lesional skin showed increased melanin content in epidermal keratinocytes but no changes in the appearance or number of m...
11 CitationsSource
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