U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination
  • The following terms were not found in MedGen: 8Hop.opcapital, Cyrillicsmall, Cyrillic.

Erythrokeratoderma en cocardes

MedGen UID:
1845700
•Concept ID:
C5848050
•
Disease or Syndrome
Synonyms: Degos 'en cocarde' erythrokeratoderma; Degos genodermatosis; Degos genodermatosis "en cocardes"; Degos genodermatosis 'en cocardes'; erythrokeratoderma "en cocardes"; erythrokeratoderma ''en cocardes''; Erythrokeratoderma ''en cocardes''; erythrokeratoderma 'en cocardes'; Erythrokeratoderma en cocarde (Degos); erythrokeratoderma en cocardes; Erythrokeratoderma en cocardes (Degos syndrome); Maladie de Degos
SNOMED CT: Erythrokeratoderma en cocardes (Degos syndrome) (239062001); Maladie de Degos (239062001); Erythrokeratoderma en cocarde (Degos) (239062001); Erythrokeratoderma en cocardes (239062001)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
•Concept ID:
C0443147
•
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Autosomal dominant inheritance (Orphanet)
 
Monarch Initiative: MONDO:0017836
Orphanet: ORPHA315

Definition

A rare, genetic, epidermal disorder characterized by intermittent (remitting and recurring), annular, polycyclic, target-like (or 'en cocardes') plaques with concentric rings of scaling erythema occurring on the extremities, flexural areas, and trunk. Concurrent erythrokeratoderma variabilis-like scaly plaques are commonly found in other parts of the body. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVErythrokeratoderma en cocardes

Recent clinical studies

Etiology

Erythrokeratoderma en cocardes.
Rajagopalan B, Pulimood S, George S, Jacob M
Clin Exp Dermatol 1999 May;24(3):173-4. doi: 10.1046/j.1365-2230.1999.00446.x. PMID: 10354172

Diagnosis

Erythrokeratodermia variabilis with erythema gyratum repens-like lesions.
Landau M, Cohen-Bar-Dayan M, Hohl D, Ophir J, Wolf CR, Gat A, Mevorah B
Pediatr Dermatol 2002 Jul-Aug;19(4):285-92. doi: 10.1046/j.1525-1470.2002.00085.x. PMID: 12220269
Erythrokeratoderma en cocardes.
Rajagopalan B, Pulimood S, George S, Jacob M
Clin Exp Dermatol 1999 May;24(3):173-4. doi: 10.1046/j.1365-2230.1999.00446.x. PMID: 10354172

Prognosis

Erythrokeratodermia variabilis with erythema gyratum repens-like lesions.
Landau M, Cohen-Bar-Dayan M, Hohl D, Ophir J, Wolf CR, Gat A, Mevorah B
Pediatr Dermatol 2002 Jul-Aug;19(4):285-92. doi: 10.1046/j.1525-1470.2002.00085.x. PMID: 12220269

Supplemental Content