Dyschromatosis universalis hereditaria with face and acral predomination : a case report and literature review

Authors

  • Sinijchaya Sahawatwong DIVISION OF DERMATOLOGY, DEPARTMENT OF MEDICINE, RAMATHIBODI HOSPITAL, MAHIDOL UNIVERSITY, BANGKOK, THAILAND
  • Natta Rajatanavin DIVISION OF DERMATOLOGY, DEPARTMENT OF MEDICINE, RAMATHIBODI HOSPITAL, MAHIDOL UNIVERSITY, BANGKOK, THAILAND

Keywords:

dyschromatosis universalis hereditaria(DUH), dyschromatosis

Abstract

Dyschromatosis universalis hereditaria (DUH) is a rare dermatologic disorder with autosomal dominant inheritance. DUH characterized by generalized, asymptomatic, dyspigmented macules of varying sizes and shapes. Facial, palm, soles and mucosal involvements are uncommon. However, those area have been described in several reports of DUH. The pathogenesis of DUH is not known but was proposed that DUH is a disorder of abnormal  melanosome synthesis rate. We report a 23-year old Thai male with generalized spotty hypopigmented and hyperpigmented macules located predominantly at face and acral area. His first degree relatives had similar dyspigmentation. This presence of truncal involvement and histopathological findings are compatible the diagnosis of with dyschromatosis universalis hereditaria. This patent is a case of DUH with atypical distributions of lesions.

References

Stuhrmann M, Hennies HC, Bukhari IA, Brakensiek K, Nurnberg G, Becker C et al. Dyschromatosis universalis hereditaria: evidence for autosomal recessive inheritance and identification of a new locus on chromosome 12q21-q23. Clin Genet 2008;73:566-72.

Griffiths WA. Reticulate pigmentary disorders--a review. Clin Exp Dermatol 1984;9:439-50.

Urabe K , Hori Y. Dyschromatosis. Semin Cutan Med Surg 1997;16:81-5.

Binitha MP, Thomas D , Asha LK. Tuberous sclerosis complex associated with dyschromatosis universalis hereditaria. Indian J Dermatol Venereol Leprol 2006;72:300-2.

Al Hawsawi K, Al Aboud K, Ramesh V , Al Aboud D. Dyschromatosis universalis hereditaria: report of a case and review of the literature. Pediatr Dermatol 2002;19:523-6.

Sethuraman G, Srinivas CR, D'Souza M, Thappa DM, Smiles L. Dyschromatosis universalis hereditaria. Clin Exp Dermatol 2002;27:477-9.

Nuber UA, Tinschert S, Mundlos S , Hauber I. Dyschromatosis universalis hereditaria: familial case and ultrastructural skin investigation. Am J Med Genet A 2004;125A:261-6.

Wang G, Li CY, Gao TW , Liu YF. Dyschromatosis universalis hereditaria: two cases in a Chinese family. Clin Exp Dermatol 2005;30:494-6.

Udayashankar C , Nath AK. Dyschromatosis universalis hereditaria: a case report. Dermatol Online J 2011;17:2.

Dhar S , Malakar S. Localized form of dyschromatosis universalis hereditaria in a 14-year-old girl. Pediatr Dermatol 1999;16:336.

Oyama M, Shimizu H, Ohata Y, Tajima S , Nishikawa T. Dyschromatosis symmetrica hereditaria (reticulate acropigmentation of Dohi): report of a Japanese family with the condition and a literature review of 185 cases. Br J Dermatol 1999;140:491-6.

Dong Y, Xiao S, Ren J, Huo J, Shi B, Wu J et al. Double-stranded RNA-specific adenosine deaminase (DSRAD) gene mutation in a Chinese family with dyschromatosis symmetrica hereditaria (DSH). Int J Dermatol 2011;50:375-8.

Suzuki N, Suzuki T, Inagaki K, Ito S, Kono M, Fukai K et al. Mutation analysis of the ADAR1 gene in dyschromatosis symmetrica hereditaria and genetic differentiation from both dyschromatosis universalis hereditaria and acropigmentatio reticularis. J Invest Dermatol 2005;124:1186-92.

El Darouti M, Marzouk SA, Fawzi M, Rabie M, El Tawdi A, Abdel Azziz M. Reticulate acropigmentation of Dohi: a report of two new associations. Int J Dermatol 2004;43:595-6.

Consigli J, Zanni MS, Ragazzini L, Danielo C. Dyschromatosis symmetrica hereditaria: report of a sporadic case. Int J Dermatol 2010;49:918-20.

Kenani N, Ghariani N, Denguezli M, Sriha B, Belajouza C , Nouira R. Dyschromatosis universalis hereditaria: two cases. Dermatol Online J 2008;14:16.

Wu CY , Huang WH. Two Taiwanese siblings with dyschromatosis universalis hereditaria. Clin Exp Dermatol 2009;34:e666-9.

Rai R, Kaur I, Handa S , Kumar B. Dyschromatosis universalis hereditaria. Indian J Dermatol Venereol Leprol 2000;66:158-9.

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Published

2026-06-11

How to Cite

Sahawatwong, S., & Rajatanavin, N. (2026). Dyschromatosis universalis hereditaria with face and acral predomination : a case report and literature review. Thai Journal of Dermatology, 27(3), 214–220. retrieved from https://he02.tci-thaijo.org/index.php/TJD/article/view/282959

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Section

Case Report