Erythrokeratoderma en cocardes Degos
| Classification according to ICD-10 | |
|---|---|
| Q82.8 | Hereditary palmoplantar keratosis |
| ICD-10 online (WHO version 2019) | |
The erythrokeratoderma en cocardes Degos is a rare congenital disease with the main feature of a Erythrokeratoderma with kokardenförmigen plaques .
Synonyms are: Degos genodermatosis "en cocardes" , Degos disease ; atypical congenital erythrokeratoderma; M. Degos; French Erythème desquamative en plaque congénital et familial Latin Genodermatosis erythematosquamosa circinata et variabilis
The disease is not to be confused with Dowling-Degos disease (Dowling-Degos disease), a rare pigment dermatosis in the joint flexor, and Degos syndrome , a rare vasculopathy .
The name refers to the first author of the first description from 1947 by Robert Degos and colleagues.
Spread and cause
The frequency is given as less than 1 in 1,000,000, inheritance is autosomal dominant . The cause is not yet known.
Clinical manifestations
Clinical criteria are:
- Circumscribed, rounded, erythematous hyperkeratotic skin changes
- Spontaneous regression with reappearance
- Localization on the trunk and extremities
- Start after birth or in early childhood
therapy
A causal treatment is currently not known.
Individual evidence
- ↑ a b Erythrokeratoderma en cocardes Degos. In: Orphanet (Rare Disease Database).
- ↑ a b c Altmeyer Encyclopedia Dermatology
- ^ Dowling-Degos disease. In: Orphanet (Rare Disease Database).
- ^ Degos syndrome. In: Orphanet (Rare Disease Database).
- ↑ R. Degos, O. Delzant, M H. Morival: erythema Desquamative en plaques, congenital et familial (genodermatosis nouvelle?) In: Bulletin de la Societe francaise de dermatology et de syphiligraphie Vol. 54, 1947, pp 442-443 .
- ^ Rare Diseases
- ^ Right Diagnosis
