Erythrokeratoderma

Classification according to ICD-10
Q82.8	Hereditary palmoplantar keratosis
ICD-10 online (WHO version 2019)

Erythrokeratodermia , "keratinizing skin reddening" (from ancient Greek ἐρυθρός erythrós , German 'red' , κέρας kéras , German 'horn' and δέρμα derma , German 'skin' ) refers to a group of congenital diseases with a cornification disorder affecting the skin of the hands and feet ( Hyperkeratosis ) together with erythroderma (reddening of the skin).

The main features are the appearance of more volatile and wandering erythemas and more persistent hyperkeratotic plaques.

This includes a number of diseases.

classification

• Erythrokeratodermia variabilis
• Erythrokeratodermia progressiva symmetrica
• Erythrokeratoderma en cocardes Degos
• Erythrokeratodermia papillaris et reticularis , not familial form, related or identical to papillomatosis confluens et reticularis . Occurrence in the 20th - 40th Age year epigastric and not affected in the neck , palms and soles of the feet.

Syndromal forms

Erythrokeratoderma can occur along with other diseases as a syndrome:

• Ataxia, spinocerebellar, type 34 (erythrokeratoderma with ataxia )
• Hypotrichosis-deafness syndrome ( hypotrichosis -Taubheits syndrome)
• Congenital non-bullous ichthyosiform erythroderma , already occurring in newborns, autosomal - recessively inherited with mutations in the ALOX12B-, ALOXE3- (both code for lipoxygenases , involved in the proliferation and specialization of skin cells) or the NIPAL4 gene in most cases
• Bullous kongenitiales ichthyosiformes Erythroderma (congenital ichthyosiform erythroderma bullosa; ichthyosiform erythroderma congenitalis Brocq; keratosis rubra congenita groove; epidermolytic hyperkeratosis, epidermolytic hyperkeratosis) with mutations at KRT1 and KRT10 on the gene locus 12q13 and 17q21-q22. General erythroderma occurring from birth with blistered skin detachment and painful hyperkeratosis.

See also

• Pityriasis rubra pilaris
• MEDNIK syndrome (Erythrokeratodermia variabilis 3; EKV3; Erythrokeratodermia variabilis of the Kamouraska type)

Individual evidence

1. ↑ Erythrokeratoderma. In: Orphanet (Rare Disease Database).
2. ^ Derma-net-online
3. ↑ ^{a b c} Altmeyer, Online Encyclopedia Dermatology
4. ↑ Ataxia, spinocerebellar, type 34. In: Orphanet (database for rare diseases).
5. ↑ Hypotrichosis-deafness syndrome. In: Orphanet (Rare Disease Database).
6. ^ Genetics Home Reference
7. ^ Right Diagnosis

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !