Livedoid vasculopathy

Summary

Livedoid vasculopathy (MONDO:0025514) is a disease and 2 clinical trials. A subtype of skin disorder — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: Unknown (Worldwide) [Orphanet-validated]
• Phenotypes (HPO): 49
• Clinical trials: 2

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	1-9 / 100 000	1	United States	Validated

Signs & symptoms

Clinical features (HPO)

49 HPO clinical features (Orphanet curated; top 49 by frequency):

HPO ID	Term	Frequency
HP:0002814	Abnormality of the lower limb	Very frequent (80-99%)
HP:0033260	Livedo racemosa	Very frequent (80-99%)
HP:0200042	Skin ulcer	Very frequent (80-99%)
HP:0000953	Hyperpigmentation of the skin	Frequent (30-79%)
HP:0000965	Cutis marmorata	Frequent (30-79%)
HP:0001058	Poor wound healing	Frequent (30-79%)
HP:0001075	Atrophic scars	Frequent (30-79%)
HP:0001581	Recurrent skin infections	Frequent (30-79%)
HP:0001903	Anemia	Frequent (30-79%)
HP:0001977	Abnormal thrombosis	Frequent (30-79%)
HP:0002619	Varicose veins	Frequent (30-79%)
HP:0002960	Autoimmunity	Frequent (30-79%)
HP:0003029	Enlargement of the ankles	Frequent (30-79%)
HP:0003565	Elevated erythrocyte sedimentation rate	Frequent (30-79%)
HP:0005339	Abnormality of complement system	Frequent (30-79%)
HP:0007572	Hyperpigmented streaks	Frequent (30-79%)
HP:0010741	Pedal edema	Frequent (30-79%)
HP:0012514	Lower limb pain	Frequent (30-79%)
HP:0025016	Abnormal capillary morphology	Frequent (30-79%)
HP:0100585	Telangiectasia of the skin	Frequent (30-79%)
HP:0100724	Hypercoagulability	Frequent (30-79%)
HP:0000819	Diabetes mellitus	Occasional (5-29%)
HP:0000822	Hypertension	Occasional (5-29%)
HP:0001760	Abnormal foot morphology	Occasional (5-29%)
HP:0001976	Reduced antithrombin III activity	Occasional (5-29%)
HP:0002160	Hyperhomocystinemia	Occasional (5-29%)
HP:0003119	Abnormal circulating lipid concentration	Occasional (5-29%)
HP:0003401	Paresthesia	Occasional (5-29%)
HP:0003493	Antinuclear antibody positivity	Occasional (5-29%)
HP:0004855	Reduced protein S activity	Occasional (5-29%)
HP:0005293	Venous insufficiency	Occasional (5-29%)
HP:0005543	Reduced protein C activity	Occasional (5-29%)
HP:0025343	Lupus anticoagulant	Occasional (5-29%)
HP:0030350	Erythematous papule	Occasional (5-29%)
HP:0031190	Superficial dermal perivascular inflammatory infiltrate	Occasional (5-29%)
HP:0031364	Ecchymosis	Occasional (5-29%)
HP:0031365	Macular purpura	Occasional (5-29%)
HP:0032018	Multiple mononeuropathy	Occasional (5-29%)
HP:0040224	Abnormality of fibrinolysis	Occasional (5-29%)
HP:0040248	Reduced plasminogen activator inhibitor 1 activity	Occasional (5-29%)
HP:0100963	Hyperesthesia	Occasional (5-29%)
HP:0410008	Abnormality of the peripheral nervous system	Occasional (5-29%)
HP:0001876	Pancytopenia	Very rare (<1-4%)
HP:0001901	Polycythemia	Very rare (<1-4%)
HP:0001974	Leukocytosis	Very rare (<1-4%)
HP:0002140	Ischemic stroke	Very rare (<1-4%)
HP:0002725	Systemic lupus erythematosus	Very rare (<1-4%)
HP:0012393	Allergy	Very rare (<1-4%)
HP:0100647	Graves disease	Very rare (<1-4%)

Identifiers

Disease identifiers

Field	Value
Canonical name	livedoid vasculopathy
Mondo ID	MONDO:0025514
MeSH	D000090122
Orphanet	542643
DOID	DOID:0040099
ICD-10-CM	L95.0
ICD-11	1237292304
SNOMED CT	238762002
UMLS	C0343081
MedGen	575376
GARD	0012784
Anatomy (UBERON)	UBERON:0003532
Is cancer (heuristic)	no

Also known as: livedo reticularis with summer ulcerations · livedo reticularis with winter ulcerations · livedo vasculitis · livedoid vasculitis · livedoid vasculopathy · segmental hyalinizing vasculopathy

Disease family

This is a subtype of skin disorder. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › integumentary system disorder › skin disorder › livedoid vasculopathy

Related subtypes (71): dermatitis, cutaneous mucinosis, skin neoplasm, pyoderma, chronic ulcer of skin, systemic sclerosis, sunburn, severe cutaneous adverse reaction, paronychia, Achenbach syndrome, erythema multiforme, erythematosquamous dermatosis, exanthem, facial dermatosis, hand dermatosis, keratosis, leg dermatosis, lichen disease, lipodystrophy, mongolian spot, reactive cutaneous fibrous lesion, rosacea, scalp dermatosis, sebaceous gland disorder, skin atrophy, skin sarcoidosis, sweat gland disorder, vesiculobullous skin disease, hyperglobulinemic purpura, ainhum, cheilitis glandularis, erythema palmare hereditarium, multiple benign circumferential skin creases on limbs, actinic prurigo, congenital lethal erythroderma, Parana hard-skin syndrome, Bazex-Dupre-Christol syndrome, nephrogenic systemic fibrosis, erosive pustular dermatosis of the scalp, pseudoxanthoma elasticum-like papillary dermal elastolysis, toxic dermatosis, oral erosive lichen, chronic actinic dermatitis, Jessner lymphocytic infiltration of the skin, acquired kinky hair syndrome, primary cutaneous plasmacytosis, cutaneous pseudolymphoma, corticosteroid-sensitive aseptic abscess syndrome, interstitial granulomatous dermatitis with arthritis, epidermal disease, skin pigmentation disorder, skin vascular disease, Wells syndrome, solar urticaria, pellagra, hereditary epidermal appendage anomaly, keratosis pilaris, dermis disorder, aquagenic pruritus, Boudhina Yedes Khiari syndrome, non-neoplastic nevus, cutaneous sclerosis, pityriasis rotunda, hematohidrosis, skin disorder caused by infection, prurigo nodularis, granuloma faciale, sclerema neonatorum, hereditary skin disorder, hand-foot syndrome, Nicolau syndrome

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 2.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	2

Top trials by phase / activity

NCT	Phase	Status	Title
NCT00975871	Not specified	COMPLETED	Correlation of Genetic Polymorphism and Livedo Vasculitis
NCT05878327	Not specified	COMPLETED	Livedoid Vasculopathy: Strong Association With Smoking, Weak Association With Thrombophilia