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Progressive Symmetric Erythrokeratodermia

Abstract

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NORD is very grateful to Gabriele Richard, MD, FACMG, Associate Scientific Director, GeneDx, Gaithersburg, Maryland, for assistance in the preparation of this report.

Synonyms of Progressive Symmetric Erythrokeratodermia

  • Darier-Gottron syndrome
  • progressive symmetric erythrokeratoderma
  • progressive symmetric erythrokeratodermia of Gottron
  • PSEK

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Progressive symmetric erythrokeratoderma (PSEK) is a rare genetic skin disorder characterized by well-demarcated plaques of reddened, dry, and thickened skin. These lesions are distributed symmetrically on the body and tend to slowly expand and progress over time. The severity and progression of the disorder can vary greatly from one person to another, even among members of the same family. PSEK is related and can be very similar in appearance to another rare disorder called erythrokeratodermia variabilis (EKV).

Organizations related to Progressive Symmetric Erythrokeratodermia

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