Orphanet Rare Disease Ontology

Last uploaded: July 3, 2024
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Preferred Name

Livedoid vasculopathy
Synonyms

Livedo reticularis with summer ulcerations

Segmental hyalinizing vasculitis

Milian atrophie blanche
Definitions

A rare vascular skin disease characterized by recurrent focal non-inflammatory thrombosis of dermal venulae, predominantly of the lower extremities, resulting in a cutaneous response manifested as pruritus and painful papules and erythematous plaques. The lesions evolve into hemorrhagic vesicles or bullae, which rupture and turn into painful ulcers merging into reticulate, confluent, geometric, and painful ulcerations. During a period of a few months, the ulcerations change to porcelain-white atrophic scars with punctate telangiectasia (so-called atrophie blanche). In active disease, lesions in different stages coexist.
ID

http://www.orpha.net/ORDO/Orphanet_542643
alternative_term

Livedo reticularis with summer ulcerations

Segmental hyalinizing vasculitis

Milian atrophie blanche
definition

A rare vascular skin disease characterized by recurrent focal non-inflammatory thrombosis of dermal venulae, predominantly of the lower extremities, resulting in a cutaneous response manifested as pruritus and painful papules and erythematous plaques. The lesions evolve into hemorrhagic vesicles or bullae, which rupture and turn into painful ulcers merging into reticulate, confluent, geometric, and painful ulcerations. During a period of a few months, the ulcerations change to porcelain-white atrophic scars with punctate telangiectasia (so-called atrophie blanche). In active disease, lesions in different stages coexist.
definition_citation

Orphanet
expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=542643
has_age_of_onset

Adolescent

Adult
hasDbXref

UMLS:C0343081

ICD-10:L95.0

MeSH:D000090122

ICD-11:EF50
label

Livedoid vasculopathy
notation

ORPHA:542643
part_of

http://www.orpha.net/ORDO/Orphanet_496924

http://www.orpha.net/ORDO/Orphanet_79379
prefixIRI

ORDO:Orphanet_542643
prefLabel

Livedoid vasculopathy
present_in

United States AND has_annual_incidence_average_value : 1.0 AND has_annual_incidence_range : 1-9 / 100 000

Worldwide AND has_point_prevalence_range : Unknown
treeView

http://www.orpha.net/ORDO/Orphanet_496924

http://www.orpha.net/ORDO/Orphanet_79379
subClassOf

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

http://www.orpha.net/ORDO/Orphanet_377792
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