loading...| Preferred Name |
Pemphigus vegetans |
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| Synonyms |
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| Definitions |
A rare autoimmune bullous skin disease characterized by mucocutaneous bullae with subsequent erosion and formation of vegetative plaques, predominantly affecting intertriginous areas and the oral mucosa. Two clinical forms of the disease are recognized: the Hallopeau type, which presents an indolent course with pustules healing as vegetative plaques and frequent lack of involvement of the oral mucosa, and the Neumann type, which takes a more severe, refractory course with vegetations developing during an eruption of vesiculobullous lesions and involvement of the oral mucosa. Serum analysis reveals antibodies against desmoglein 1 and 3. |
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| ID |
http://www.orpha.net/ORDO/Orphanet_79479 |
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| definition |
A rare autoimmune bullous skin disease characterized by mucocutaneous bullae with subsequent erosion and formation of vegetative plaques, predominantly affecting intertriginous areas and the oral mucosa. Two clinical forms of the disease are recognized: the Hallopeau type, which presents an indolent course with pustules healing as vegetative plaques and frequent lack of involvement of the oral mucosa, and the Neumann type, which takes a more severe, refractory course with vegetations developing during an eruption of vesiculobullous lesions and involvement of the oral mucosa. Serum analysis reveals antibodies against desmoglein 1 and 3. |
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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=79479 |
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| has_age_of_onset |
All ages |
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| hasDbXref |
ICD-11:EB40.0Y ICD-10:L10.1 UMLS:C0263316 MedDRA:10057053 |
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| label |
Pemphigus vegetans |
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| notation |
ORPHA:79479 |
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| part_of | ||
| prefixIRI |
ORDO:Orphanet_79479 |
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| prefLabel |
Pemphigus vegetans |
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| present_in |
Germany AND has_point_prevalence_average_value : 0.391 AND has_point_prevalence_range : 1-9 / 1 000 000 |
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| treeView | ||
| subClassOf |