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      A case of dyschromatosis universalis hereditaria with adermatoglyphia: A rare association

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          Abstract

          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.

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          Most cited references 15

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          A gene locus responsible for dyschromatosis symmetrica hereditaria (DSH) maps to chromosome 6q24.2-q25.2.

          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 [Z(max)] = 7.28 at recombination fraction [theta] = 0.00) and pedigree B (Z(max) = 2.41 at theta = 0.00) mapped the locus for DSH in the two families to chromosome 6q. Subsequent multipoint analysis of the two families also provided additional support for the DSH gene being located within the region 6q24.2-q25.2, with Z(max) = 10.64. Haplotype analysis confined the locus within an interval of 10.2 Mbp, flanked by markers D6S1703 and D6S1708. The two families had no identical haplotype within the defined region, which suggests that the two families were different in origin. Further work on identification of the gene for DSH will open new avenues to exploration of the genetics of pigmentation.
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            Generalized Dowling-Degos disease.

            Dowling-Degos disease (DDD) is a rare inherited disease characterized by reticular hyperpigmentation on flexor surfaces. We sought to describe several cases of generalized DDD, a presentation that resemble dyschromatosis universalis hereditaria. The clinical manifestations, histopathologic, and genetic studies of a family with autosomal dominant inheritance were analyzed. The father and his sister had reticular hyperpigmentation on flexor surfaces, whereas the daughter and son had generalized hyperpigmentation with numerous hypopigmented or erythematous macules and papules on the trunk and limbs. Skin biopsy specimens from both types of lesions all had typical features of DDD. Biopsy specimens from axillary skin had features of Galli-Galli disease, an acantholytic form. There were no mutations of the double-stranded RNA-specific adenosine deaminase or keratin 5 genes. Generalizations cannot be drawn from genetic study of only one family. DDD can present with generalized hyperpigmentation and hypopigmented papules.
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              Dyschromatosis universalis hereditaria: report of a case and review of the literature.

              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.
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                Author and article information

                Journal
                Indian Dermatol Online J
                Indian Dermatol Online J
                IDOJ
                Indian Dermatology Online Journal
                Medknow Publications & Media Pvt Ltd (India )
                2229-5178
                2249-5673
                Mar-Apr 2015
                : 6
                : 2
                : 105-109
                Affiliations
                Department of Skin and VD, Guru Gobind Singh Medical College, Faridkot, Punjab, India
                Author notes
                Address for correspondence: Dr. Pritish Bhoyar, Department of Skin and VD, Guru Gobind Singh Medical College, Faridkot, Punjab, India. E-mail: pritish_bhoyar2000@ 123456yahoo.com
                Article
                IDOJ-6-105
                10.4103/2229-5178.153013
                4375753
                Copyright: © Indian Dermatology Online Journal

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                Categories
                Case Report

                Dermatology

                adermatoglyphia, dyschromatosis universalis hereditaria, reticulate pigmentation

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