Orphanet Rare Disease Ontology

Last uploaded: July 3, 2024
Preferred Name

IgA pemphigus

Synonyms
Definitions

A rare autoimmune bullous skin disease characterized by painful and pruritic vesiculopustular eruptions resulting from circulating IgA antibodies against keratinocyte cell surface components. The lesions are typically found at the periphery of erythematous annular plaques and favor intertriginous regions. Histologically and immunologically, IgA pemphigus can be subdivided into subcorneal pustular dermatosis and intraepidermal neutrophilic IgA dermatosis.

ID

http://www.orpha.net/ORDO/Orphanet_555905

definition

A rare autoimmune bullous skin disease characterized by painful and pruritic vesiculopustular eruptions resulting from circulating IgA antibodies against keratinocyte cell surface components. The lesions are typically found at the periphery of erythematous annular plaques and favor intertriginous regions. Histologically and immunologically, IgA pemphigus can be subdivided into subcorneal pustular dermatosis and intraepidermal neutrophilic IgA dermatosis.

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=555905

has_age_of_onset

All ages

hasDbXref

ICD-11:EB40.Y

ICD-10:L10.8

UMLS:C1274167

label

IgA pemphigus

notation

ORPHA:555905

part_of

http://www.orpha.net/ORDO/Orphanet_79669

prefixIRI

ORDO:Orphanet_555905

prefLabel

IgA pemphigus

present_in

Worldwide AND has_point_prevalence_range : Unknown

treeView

http://www.orpha.net/ORDO/Orphanet_79669

subClassOf

http://www.orpha.net/ORDO/Orphanet_377788

http://www.orpha.net/ORDO/Orphanet_557493

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