" /> Diffuse cutaneous mastocytosis - CISMeF





ICD-11 code : XH2RG8;

Preferred Label : Diffuse cutaneous mastocytosis;

ICD-11 definition : Diffuse cutaneous mastocytosis is a rare form of cutaneous mastocytosis in which mast cells infiltrate the entire skin diffusely. It usually presents in the neonatal period and is characterized by erythroderma, a doughy ''peau d'orange'' appearance and blistering after minor trauma. Affected infants are at risk of systemic disease and severe complications including anaphylaxis and diarrhoea. The condition may resolve spontaneously.;

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Diffuse cutaneous mastocytosis is a rare form of cutaneous mastocytosis in which mast cells infiltrate the entire skin diffusely. It usually presents in the neonatal period and is characterized by erythroderma, a doughy ''peau d'orange'' appearance and blistering after minor trauma. Affected infants are at risk of systemic disease and severe complications including anaphylaxis and diarrhoea. The condition may resolve spontaneously.

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30/07/2025


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