Synonym(s)
Erythrodermia congenitalis progressive symmetrica; Erythro- et Keratodermia figurata variabilis; Erythrokeratoderma congenitum progressivum symmetric; Erythrokeratodermia verrucosa progressive; Gottron syndrome II; Keratodermia symmetrica progressive; Progressive symmetric erythrokeratoderma; PSEK
HistoryThis section has been translated automatically.
Darier, 1911; Gottron, 1922
DefinitionThis section has been translated automatically.
Autosomal-dominantly inherited cornification anomaly with incomplete penetrance and variable expressivity. In many cases the identity with the erythrokeratodermia figurata variabilis is discussed (occurrence of both disease patterns in one family).
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EtiopathogenesisThis section has been translated automatically.
mutation in the LORICRIN gene on chromosome 1q21 have been discussed for this clinical picture. However, there is a lack of confirmation for this mutation.
ManifestationThis section has been translated automatically.
Early youth to early adulthood.
LocalizationThis section has been translated automatically.
Extremities.
Clinical featuresThis section has been translated automatically.
Flat, sharply defined, partly reddened, partly pigmented, non follicularly bound keratoses. Isomorphic irritant effect.
HistologyThis section has been translated automatically.
Uncharacteristic round cell dermal infiltrates, acanthosis with hypergranulose.
Differential diagnosisThis section has been translated automatically.
Erythrokeratodermia figurata variabilis; pityriasis rubra pilaris; Netherton syndrome as well as the entire spectrum of non-bladder-forming congenital ichthyoses.
TherapyThis section has been translated automatically.
Mild keratolytic ointments such as 3% salicylvaseline (e.g. Salicylvaseline Lichtenstein, R228 ) as well as salt baths (e.g. 1% table salt or with salt from the Dead Sea) or tar baths (e.g. Ichtho bath). Try with 2% Ichthyol cream (e.g. Ichthoderm). If necessary try with retinoids, externally (e.g. tazarotene) or internally (e.g. neotigasone).
LiteratureThis section has been translated automatically.
- Arroyo MP (2002) A young boy with symmetric hyperkeratotic plaques: progressive symmetric erythrokeratoderma (PSEK). J Drugs Dermatol 1: 326-328
- Chu DH et al (2003) Progressive and symmetric erythrokeratoderma. Dermatol Online J 9: 21
- Darier J (1911) Erythrokeratoderma verruqueuse en nappes, symetrique et progressive. Bull Soc Fr Dermatol Syphil 22: 252-264
- Emmert S et al (1998) Erythrokeratodermia progressiva symmetrica Darier-Gottron with generalized expression. dermatologist 49: 666-671
- Frank J, christiano AM (2001) The cornified envelope. In: Barker J, MacGrath J (eds) Cell adhesion and migration in skin disease, vol. 8 Haarwood academic publishers, pp 9-26
- Gottron HA (1922) Congenital symmetrical progressive erythrokeratoderma. Z Skin and venereal disease 4: 493-494
- Hopsu-Havu VK et al (1971) Erythrokeratodermia congenitalis progressiva symmetrica (Gottron). Dermatologica 142: 137-144
- Ruiz-Maldonado R et al (1982) Erythrokeratodermia progressiva symmetrica. Dermatologica 164: 133-141
- van Steensel M (2004) Does progressive symmetric erythrokeratoderma exist? Br J Dermatol 150: 1043-1045
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Incoming links (9)
Erythro- et keratodermia figurata variabilis; Erythrokeratoderma congenitum progressivum symmetric; Erythrokeratodermia figurata variabilis; Erythrokeratodermia progressive partim symmetrica; Erythrokeratodermia verrucosa progressive; Gottron syndrome ii; Keratodermia symmetrica progressive; Olmsted syndrome; Salicylic acid ointment 1/2/3/5/10 or 20% (nrf 11.43.);Outgoing links (8)
Acitretin; Erythrokeratodermia figurata variabilis; LOR Gene; Netherton syndrome; Pityriasis rubra pilaris (adult type); Retinoids; Salicylic acid ointment 1/2/3/5/10 or 20% (nrf 11.43.); Tazarotene;Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
Prof. Dr. med. Peter Altmeyer
