loading...| Preferred Name |
Diffuse cutaneous mastocytosis |
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| Synonyms |
Diffuse cutaneous maculopapulous mastocytosis |
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| Definitions |
Diffuse cutaneous mastocytosis (DCM) is a rare form of cutaneous mastocytosis (CM; see this term) characterized by generalized erythroderma, various degrees of blistering, skin with a ''peau d'orange'' appearance and the accumulation of mast cells in the skin. At least two DCM variants are recognized, one with extreme blistering (Bullous DCM; see this term) and one with infiltrations (Pseudoxanthomatous DCM; see this term). |
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| ID |
http://www.orpha.net/ORDO/Orphanet_79456 |
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| alternative_term |
Diffuse cutaneous maculopapulous mastocytosis DCM |
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| definition |
Diffuse cutaneous mastocytosis (DCM) is a rare form of cutaneous mastocytosis (CM; see this term) characterized by generalized erythroderma, various degrees of blistering, skin with a ''peau d'orange'' appearance and the accumulation of mast cells in the skin. At least two DCM variants are recognized, one with extreme blistering (Bullous DCM; see this term) and one with infiltrations (Pseudoxanthomatous DCM; see this term). |
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| definition_citation |
Orphanet |
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| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=79456 |
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| has_age_of_onset |
Infancy Neonatal |
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| has_inheritance |
Not applicable |
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| hasDbXref |
ICD-11:XH2RG8 UMLS:C0024901 MedDRA:10012812 ICD-11:2A21.1Y ICD-10:Q82.2 |
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| label |
Diffuse cutaneous mastocytosis |
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| notation |
ORPHA:79456 |
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| part_of | ||
| prefixIRI |
ORDO:Orphanet_79456 |
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| prefLabel |
Diffuse cutaneous mastocytosis |
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| present_in |
Worldwide AND has_cases/families_value : 30.0 (Case) Worldwide AND has_point_prevalence_range : <1 / 1 000 000 |
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| treeView | ||
| subClassOf |