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      Generalized Dowling-Degos disease

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      Journal of the American Academy of Dermatology
      Elsevier BV

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          Abstract

          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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          Author and article information

          Journal
          Journal of the American Academy of Dermatology
          Journal of the American Academy of Dermatology
          Elsevier BV
          01909622
          August 2007
          August 2007
          : 57
          : 2
          : 327-334
          Article
          10.1016/j.jaad.2006.12.023
          17637446
          055226b2-9bde-4c88-ae00-b6bf3949037b
          © 2007

          https://www.elsevier.com/tdm/userlicense/1.0/

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