" /> Epidermolysis bullosa simplex with mottled pigmentation - CISMeF





Preferred Label : Epidermolysis bullosa simplex with mottled pigmentation;

ICD-11 definition : Epidermolysis bullosa simplex with mottled pigmentation (EBS-MP) is an autosomal dominant basal subtype of EBS due to mutations in the KRT5 gene which encodes keratin 5. It is characterized by generalised blistering with mottled or reticulate brown pigmentation.;

ICD-11 synonym : EBS with mottled pigmentation; EBS - [epidermolysis bullosa simplex] with mottled pigmentation; EBS-MP - [epidermolysis bullosa simplex with mottled pigmentation];

ICD-11 acronym : EBS-MP;

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Epidermolysis bullosa simplex with mottled pigmentation (EBS-MP) is an autosomal dominant basal subtype of EBS due to mutations in the KRT5 gene which encodes keratin 5. It is characterized by generalised blistering with mottled or reticulate brown pigmentation.

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05/05/2025


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