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D. Steinmann, J. Bergmann, H. Kaltofen

Epidermolysis bullosa

Epidermolysis bullosa

Schlüsselwörter Epidermolysis bullosa; ICD 10: Q81; trauma-induced blister formation of the skin and mucosa
Keywords Epidermolysis bullosa; ICD 10: Q81; trauma-induced blister formation of the skin and mucosa
Zusammenfassung Dieser Beitrag enthält keine Zusammenfassung
Summary

Epidermolysis bullosa (EB) is a heterogeneous group of inherited rare diseases, which are characterized by trauma-induced blister formation of the skin and mucosa. The underlying cause is a functional deficiency of structural proteins of the dermo-epidermal junction. Depending on the level of the blister formation, EB is divided into EB simplex (intra- epidermal), junctional EB (within the lamina lucida), dystrophic EB (below the lamina lucida) and Kindler syndrome (variable level of split formation). Besides different distinct blister formation and pain symptoms secondary problems like anaemia, oesophageal stenosis, cardiomyopathy or squamous cell carcinoma may occur. Furthermore, many patients present with severe microstomia and ankyloglosson as well as significant dental decay.

Since causal therapies are not available, strict prevention of friction and trauma is essential to avoid blister formation.

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