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Erythrokeratodermia variabilis et progressiva

Other Names for this Disease
  • Darier-Gottron disease
  • EKV
  • EKVP
  • Erythrokeratodermia variabilis
  • Erythrokeratodermia variabilis, Mendes da Costa type
More Names
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Erythrokeratodermia variabilis et progressiva is a skin condition characterized by well-defined round or oval red scaly patches that may join together to form map-like patterns. Some patches are fixed, occurring most often on the outer surfaces of the arms and legs, while others are migratory - lasting for hours to days and then fading or moving to another location. Some skin lesions are accompanied by burning or itching sensations. Common triggers include emotional stress, temperature changes, mechanical friction and hot or cold weather. Skin lesions often occur during the fist year of life, gradually progress during childhood, and then stabilize during puberty.[1] Treatment is aimed at alleviating symptoms and may include topical retinoids or antihistamines.[2]  
Last updated: 5/21/2012


  1. Abeyakirthi S. Erythrokeratoderma. DermNet NZ. July 1, 2011; Accessed 5/21/2012.
  2. Richard G. Erythrokeratodermia Variabilis Treatment & Management. Medscape Reference. January 24, 2012; Accessed 5/21/2012.
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Basic Information

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
  • Genetics Home Reference (GHR) contains information on Erythrokeratodermia variabilis et progressiva. This website is maintained by the National Library of Medicine.

In Depth Information

  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • The Online Mendelian Inheritance in Man (OMIM) is an catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Erythrokeratodermia variabilis et progressiva. Click on the link to view a sample search on this topic.