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Darier Disease

An autosomal dominantly inherited skin disorder characterized by warty malodorous papules that coalesce into plaques. It is caused by mutations in the ATP2A2 gene encoding SERCA2 protein, one of the SARCOPLASMIC RETICULUM CALCIUM-TRANSPORTING ATPASES. The condition is similar, clinically and histologically, to BENIGN FAMILIAL PEMPHIGUS, another autosomal dominant skin disorder. Both diseases have defective calcium pumps (CALCIUM-TRANSPORTING ATPASES) and unstable desmosomal adhesion junctions (DESMOSOMES) between KERATINOCYTES.

Year introduced: 2009 (1966)

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Tree Number(s): C16.320.850.190, C17.800.428.275, C17.800.827.190

MeSH Unique ID: D007644

Entry Terms:

  • Disease, Darier
  • Darier's Disease
  • Dariers Disease
  • Disease, Darier's
  • Keratosis Follicularis
  • Darier-White Disease
  • Darier White Disease
  • Darier-White Diseases
  • Disease, Darier-White
  • Diseases, Darier-White
  • Acrokeratosis Verruciformis of Hopf
  • Hopf Acrokeratosis Verruciformis
  • Hopf Disease
  • Disease, Hopf
  • Diseases, Hopf
  • Hopf Diseases
  • Acrokeratosis Verruciformis
  • Verruciformis, Acrokeratosis
  • Acantholytic Dyskeratotic Epidermal Nevus
  • Acantholytic Dyskeratotic Epidermal Nevi

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