Erythème desquamative en plaque congénital et familialQ82.8
Synonym(s)
atypical congenital erythrokeratoderma; Degos Disease; Degos M.; Erythrokeratoderma congenital; Erythrokeratoderma en cocardes; Genodermatosis erythematosquamosa circinata et variabilis; Mr. Degos
HistoryThis section has been translated automatically.
Degos et al., 1947
DefinitionThis section has been translated automatically.
Rare, atypical, hereditary erythrokeratoderma with characteristic coccardium-shaped plaques on the lower extremities. Some authors consider the disease to be a variant of erythrokeratodermia figurata variabilis.
EtiopathogenesisThis section has been translated automatically.
Unknown. Autosomal dominant inheritance is described in some families.
ManifestationThis section has been translated automatically.
At birth or in infancy.
LocalizationThis section has been translated automatically.
Lower extremity.
Clinical featuresThis section has been translated automatically.
Cockade-like, centrally scaled erythema slices. The expression is variable, from strongly reddened to regression within a few weeks and recurrence in loco. Hyperkeratosis on the knees.
HistologyThis section has been translated automatically.
Non-specific mucin deposition.
Differential diagnosisThis section has been translated automatically.
Tinea corporis, anular erythema.
TherapyThis section has been translated automatically.
Unknown.
Progression/forecastThis section has been translated automatically.
Recurrent course, improvement and healing possible in summer.
LiteratureThis section has been translated automatically.
- Degos R, Delzant O, Morival MH (1947) Erythema desquamative en plaques, congenital et familial (genodermatosis nouvelle?). Bull Soc Fr Dermatol Syphilol 54: 442-443
- Landau M et al (2002) Erythrokeratodermia variabilis with erythema gyratum repens-like lesions. Pediatric dermatol 19: 285-292
- Rajagopalan B et al (1999) Erythrokeratoderma en cocardes. Clin Exp Dermatol 24: 173-174