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Epidermolysis bullosa simplex localized (Weber-Cockayne)Q81.0

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 06.01.2022

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Synonym(s)

acral epidermolysis bullosa simplex; Epidermolysis bullosa manuum et pedum aestivalis; Epidermolysis bullosa simplex Type Weber-Cockayne; Localized epidermolysis bullosa simplex; localized epidermolysis simplex; palmoplantar epidermolysis bullosa simplex; recurrent bullous eruption of the hands and the feet; summer eruption of the feet; Weber-Cockayne Syndrome

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HistoryThis section has been translated automatically.

Elliot 1895; Weber 1926; Cockayne 1938

DefinitionThis section has been translated automatically.

Most frequent (75-85% of all EB cases), clinically mild, autosomal-dominantly inherited epidermolysis bullosa with blistering as a consequence of relatively minor traumatic stress on hands and feet (blister craving).

Occurrence/EpidemiologyThis section has been translated automatically.

The incidence of localized EBS is 1:30,000 to 1:50,000.

EtiopathogenesisThis section has been translated automatically.

Autosomal dominant inheritance. It is based on mutations in the keratin genes KRT 5 (gene locus: 12q13) and KRT14 (gene locus: 17q12-q21).

Another localized form of autosomal dominant inherited epidermolysis bullosa simplex lsit characterized by a mutation in the ITGB4 gene (Identical phenotype/different genotype). ITGB4 codes for integrin beta 4 [see integrins below] (gene location: 17q11-qter).

Both mutations lead to identical disorders in the keratin filament network(cytoskeleton) of basal keratinocytes.

The blister is intraepithelial and thus bursts more easily than a normal marsh blister.

ManifestationThis section has been translated automatically.

In early childhood (when children learn to walk) or adolescence.

LocalizationThis section has been translated automatically.

Locally limited to Palmae and Plantae.

Clinical featuresThis section has been translated automatically.

Particularly in summer, acute blisters and erosions on the feet after marches and sports activities, less frequently on the hands, without impairment of the general condition. The plantar "marching blisters" occur particularly at higher ambient temperatures, but can be completely absent in winter. They are usually tightly stretched, but burst relatively easily due to their intraepithelial position (thus only thin bubble cover), so that often only extensive erosions are visible.

Further accompanying symptoms:

  • Often hyperhidrosis of the feet.
  • Mild palmoplantar keratoses, often also in mechanically stressed areas.
  • No extracutaneous manifestations.

HistologyThis section has been translated automatically.

Epidermolytic suprabasal bladder.

Differential diagnosisThis section has been translated automatically.

Epidermolysis bullosa group

Complication(s)This section has been translated automatically.

Bacterial superinfection of the blisters or erosions.

External therapyThis section has been translated automatically.

Avoid mechanical irritation. No tight and concluding footwear, as hyperhidrosis and mechanical irritation are the best conditions for blister formation. Blisters should be opened and treated with antiseptic solutions such as quinolinol solution(e.g. Chinosol 1:1000), R042, potassium permanganate solution (light pink) or polyvidon iodine compounds (e.g. Braunovidon solution, ointment) to avoid secondary infections. Healing is usually without complications and scars.

Progression/forecastThis section has been translated automatically.

Good, but lifelong tendency to blistering; with increasing age, marked improvement in blistering

LiteratureThis section has been translated automatically.

  1. Cockayne EA (1938) Recurrent bullous eruption of the feet. Brit J Derm Syph 50: 358-362
  2. Elliot T (1895) Two cases of epidermolysis bullosa. Journal of Cutaneous and Genitourinary Diseases (Chicago) 13: 10-18
  3. Fine JD et al (2000) Revised classification system for inherited epidermolysis bullosa: Report of the Second International Consensus Meeting on diagnosis and classification of epidermolysis bullosa. J Am Acad Dermatol 42:1051-1066
  4. Fine JD et al (2008) The classification of inherited epidermolysis bullosa (EB): Report of the Third International Consensus Meeting on Diagnosis and Classification of EB. J Am Acad Dermatol 58:931-950
  5. Cho JW et al (2014) Weber-Cockayne Type Epidermolysis Bullosa Simplex Resulting from a Novel Mutation (c. 608T>C) in the Keratin 5 genes. Ann Dermatol 26: 739-742.
  6. Fivenson DP (2003) Graftskin therapy in epidermolysis bullosa. J Am Acad Dermatol 48: 886-892
  7. Horn HM (2000) The clinical spectrum of epidermolysis bullosa simplex. Br J Dermatol 142: 468-472
  8. Muller FB (1998) Novel K5 and K14 mutations in German patients with the Weber-Cockayne variant of epidermolysis bullosa simplex. J Invest Dermatol 111: 900-902
  9. Weber FP (1926) Recurrent bullous eruption of the feet in a child. Proceedings of the Royal Society of Medicine (London) 19: 72

Authors

Last updated on: 06.01.2022

Author: Prof. Dr. med. Peter Altmeyer