Orphanet Rare Disease Ontology

Last uploaded: December 13, 2023
Preferred Name

Discoid lupus erythematosus

Synonyms
Definitions

A rare form of chronic cutaneous lupus erythematosus characterized by erythematous, scaly papules and plaques preferentially occurring on sun-exposed skin areas (scalp, face, and ears) and exhibiting follicular plugging, pigmentary changes, and central atrophy, scarring, and telangiectasia. Skin biopsy shows a perivascular and periadnexal lymphocytic infiltrate and involvement of the dermoepidermal junction with thickening of the basement membrane and vacuolar degeneration of the basal cells. A small percentage of patients may develop systemic lupus erythematosus.

ID

http://www.orpha.net/ORDO/Orphanet_90281

definition

A rare form of chronic cutaneous lupus erythematosus characterized by erythematous, scaly papules and plaques preferentially occurring on sun-exposed skin areas (scalp, face, and ears) and exhibiting follicular plugging, pigmentary changes, and central atrophy, scarring, and telangiectasia. Skin biopsy shows a perivascular and periadnexal lymphocytic infiltrate and involvement of the dermoepidermal junction with thickening of the basement membrane and vacuolar degeneration of the basal cells. A small percentage of patients may develop systemic lupus erythematosus.

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=90281

has_age_of_onset

Adolescent

Adult

Childhood

Elderly

hasDbXref

ICD-11:EB51.0

MedDRA:10013072

MeSH:D008179

UMLS:C5574816

ICD-10:L93.0

label

Discoid lupus erythematosus

notation

ORPHA:90281

part_of

http://www.orpha.net/ORDO/Orphanet_163531

prefixIRI

ORDO:Orphanet_90281

prefLabel

Discoid lupus erythematosus

present_in

United States AND has_annual_incidence_average_value : 3.56 AND has_annual_incidence_range : 1-9 / 100 000

Sweden AND has_annual_incidence_average_value : 3.2 AND has_annual_incidence_range : 1-9 / 100 000

treeView

http://www.orpha.net/ORDO/Orphanet_163531

subClassOf

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

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

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