title: Generalized Dowling-Degos disease.
abstract: BACKGROUND
  Dowling-Degos disease (DDD) is a rare inherited disease characterized by reticular hyperpigmentation on flexor surfaces.
  
  
  OBJECTIVE
  We sought to describe several cases of generalized DDD, a presentation that resemble dyschromatosis universalis hereditaria.
  
  
  METHODS
  The clinical manifestations, histopathologic, and genetic studies of a family with autosomal dominant inheritance were analyzed.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  Generalizations cannot be drawn from genetic study of only one family.
  
  
  CONCLUSIONS
  DDD can present with generalized hyperpigmentation and hypopigmented papules.
authors.affiliation: Department of Dermatology, Mackay Memorial Hospital, Taipei, Taiwan. yuhung_wu@yahoo.com
authors.forename: Yu-Hung
authors.initials: YH
authors.lastname: Wu
authors.forename: Yang-Chih
authors.initials: YC
authors.lastname: Lin
mesh_terms: D000051:Acantholysis / Q000473:pathology; D000243:Adenosine Deaminase / Q000235:genetics; D000328:Adult; D001365:Axilla; D005260:Female; D005799:Genes, Dominant; D006801:Humans; D017495:Hyperpigmentation / Q000235:genetics* / Q000473:pathology*; D017496:Hypopigmentation / Q000235:genetics / Q000473:pathology; D053555:Keratin-5 / Q000235:genetics; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D010859:Pigmentation Disorders / Q000473:pathology; D016601:RNA-Binding Proteins; D012867:Skin / Q000473:pathology
chemical_list: D053555:Keratin-5; D016601:RNA-Binding Proteins; C567955:ADARB1 protein, human; D000243:Adenosine Deaminase