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Dyschromatosis universalis hereditaria in a young Nigerian female

Published on Jul 1, 2009in International Journal of Dermatology1.794
· DOI :10.1111/j.1365-4632.2009.03290.x
Sm Yusuf5
Estimated H-index: 5
,
Muhammad S Mijinyawa4
Estimated H-index: 4
+ 1 AuthorsAz Mohammed12
Estimated H-index: 12
Abstract
Dyschromatosis universalis hereditaria (DUH) is a clinically heterogeneous disorder that shows generalized mottled pigmentation. It occurs most commonly in Japanese persons, with sporadic reports from South Africa, India, and Iraq. Histopathology reveals a variable degree of pigmentary incontinence. Although the precise etiology of this disorder is not yet known, the clinicopathological findings implicate an inherent abnormality of melanosomes or melanin processing. We describe a case in a young Nigerian girl.
  • References (9)
  • Citations (5)
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References9
Newest
#1Iqbal A. Bukhari (King Faisal University)H-Index: 9
Last. M. StuhrmannH-Index: 2
view all 3 authors...
18 CitationsSource
#1Qinghe Xing (CAS: Chinese Academy of Sciences)H-Index: 27
#2Xiangdong ChenH-Index: 3
Last. Lin He (CAS: Chinese Academy of Sciences)H-Index: 57
view all 11 authors...
Dyschromatosis symmetrica hereditaria (DSH) is a hereditary skin disease characterized by the presence of hyperpigmented and hypopigmented macules on extremities and face. The gene, or even its chromosomal location, for DSH has not yet been identified. In this study, two Chinese families with DSH were identified and subjected to a genomewide screen for linkage analysis. Two-point linkage analysis for pedigree A (maximum LOD score [Zmax] = 7.28 at recombination fraction [θ] = 0.00) and pedigree B...
42 CitationsSource
#1Khalid Al HawsawiH-Index: 2
#2Khalid Al AboudH-Index: 2
Last. Daifullah Al Aboud (King Khalid University)H-Index: 1
view all 4 authors...
We describe dyschromatosis universalis in a 19-month-old Saudi Arabian girl. She had no associated defects and none of the other family members were affected. Similar cases reported from countries other than the Far East, where the disease was first described, are discussed.
33 CitationsSource
Dyschromatosis universalis hereditaria is a common disorder in Japan. We report a case in an Indian woman with no family history of the disorder.
5 Citations
#1Nam Soo Kim (Ajou University)H-Index: 4
#2Sungbin Im (Ajou University)H-Index: 2
Last. Soo-Chan Kim (Ajou University)H-Index: 24
view all 3 authors...
We report electron microscopic findings from both hyperchromic and achromic macules of dyschromatosis universalis hereditaria (DUH). The keratinocytes of the hyperchromic macules contained numerous, fully melanized melanosomes; almost all of them were aggregated to form the melanosome complex. In contrast, the melanosomes were absent from both keratinocytes and melanocytes of achromic macules, even though intact melanocytes were found. Our results suggest that DUH may be a disorder of melanosome...
21 CitationsSource
A case of dyschromatosis universalis hereditaria is reported in a 16-year-girl. She had asymptomatic spotty, depigmented and hyperpigmented macules distributed bilaterally all over the body, sparing the palms and soles. She also had associated grandmal epilepsy since early childhood. The common origin of epidermis and brain from ectoderm suggests that the association of epilepsy and dyschromatosis universalis hereditaria in this patient is more than fortuitous.
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#1R.J.G. Rycroft (St. John's Hospital)H-Index: 26
#2C.D. Calnan (St. John's Hospital)H-Index: 5
Last. R.S. Wells (Guy's Hospital)H-Index: 5
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Summary A syndrome of hyper- and hypopigmentation with melanin, small stature and high-tone deafness is described in the 6-year-old daughter of a first cousin marriage. The condition is discussed in relation to other similar inherited dyschromatoses.
25 CitationsSource
1. Two familial cases of dyschromatosis universalis hereditaria are reported. 2. In the same family the occurrence of similar dermatoses is noted in five generations. 3. Unexposed parts of the body is as strongly affected as the exposed area. And even the palm and sole are involved. 4. The pigmentary disturbance is caused by genotype with epistases of some other factors than photosensitivity. 5. Thus the relationship with dyschromatosis symmetrica hereditaria is rejected in spite of clinical res...
25 CitationsSource
Cited By5
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#1Aayush GuptaH-Index: 11
#2Yugal K SharmaH-Index: 5
Last. Archana SinghH-Index: 57
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© 2015 The Authors. doi: 10.2340/00015555-2030 Journal Compilation © 2015 Acta Dermato-Venereologica. ISSN 0001-5555 The dyschromatoses are a group of rare genodermatoses characterised by the presence of asymptomatic mottled hyperpigmented macules admixed with variably sized hypopigmented macules (1). Dyschromatoses are divided into dyschromatosis symmetrica hereditaria (DSH) and dyschromatosis universalis hereditaria (DUH). DUH is clinically diagnosed on the basis of widely distributed small hy...
3 CitationsSource
#1Ying-Xia CuiH-Index: 11
#2Xin-Yi XiaH-Index: 11
Last. Xiao-Jun LiH-Index: 9
view all 14 authors...
Objective Dyschromatosis universalis hereditaria (DUH) is a rare heterogeneous pigmentary genodermatosis, which was first described in 1933. The genetic cause has recently been discovered by the discovery of mutations in ABCB6. Here we investigated a Chinese family with typical features of autosomal dominant DUH and 3 unrelated patients with sporadic DUH.
13 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
#1Yi-Ying Chin (KMU: Kaohsiung Medical University)H-Index: 3
#2Gwo-Shing Chen (KMU: Kaohsiung Medical University)H-Index: 25
Last. Cheng-Che E. Lan (KMU: Kaohsiung Medical University)H-Index: 21
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3 CitationsSource