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arterial dissection-lentiginosis syndrome - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

arterial dissection-lentiginosis syndrome

MONDO:0010889

Also known as:arterial dissection with lentiginosis

Overview

Arterial dissection-lentiginosis is a rare association syndrome, reported in several members of two families to date, and characterized by arterial dissection, occurring at an early age and presenting with a range of manifestations depending on the vascular territory involved (ex. headache, dysphasia, hemiparesis), in association with cystic medial necrosis and multiple lentigines (brown and black in color and mainly affecting the skin of the trunk and extremities).

GeneticsAutosomal recessive

Clinical Presentation

Features include: Multiple lentigines, Cystic medial necrosis, and Arterial dissection.

Explore clinical features

Diagnosis

Biomarker and diagnostic research for arterial dissection-lentiginosis syndrome has been reported in the published literature.

Prognosis

Estimated prevalence: <1 in 1,000,000 (VERY_RARE).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for arterial dissection-lentiginosis syndrome.

201 publications have been identified in PubMed for arterial dissection-lentiginosis syndrome. Kisho has analyzed 97 by research type. Research spans Review / Meta-Analysis (49%), Case Report / Case Series (22%), and Epidemiology / Natural History (11%).

Research Type	Count	% of Total
Research summaries	48	49%

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 6:58 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:600459

Orphanet

European rare disease database

Orphanet:1682

GARD

Genetic and Rare Diseases Info Center

GARD:16577

Questions for Your Care Team

→Should my partner and I meet with a genetic counselor to discuss carrier status and family planning?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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arterial dissection-lentiginosis syndrome

Overview

Clinical Presentation

Explore clinical features

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

OMIM

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

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