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Livedo racemosa

MedGen UID:
1778101
Concept ID:
C5441660
Sign or Symptom
Synonyms: Livedo Racemosa; Racemosa, Livedo
 
HPO: HP:0033260

Definition

Livedo racemosa describes a reddish-blue mottling of the skin in an irregular, reticular pattern. It differs from the more common livedo reticularis by its shape. Livedo racemosa consists of broken circular segments resulting in a seemingly larger pattern, as opposed to the fine, regular, complete network of livedo reticularis. Livedo racemosa results from permanent impairment of peripheral blood flow and, unlike livedo reticularis, it persists on warming. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLivedo racemosa

Conditions with this feature

Sneddon syndrome
MedGen UID:
76449
Concept ID:
C0282492
Disease or Syndrome
Sneddon syndrome is a noninflammatory arteriopathy characterized by onset of livedo reticularis in the second decade and onset of cerebrovascular disease in early adulthood (summary by Bras et al., 2014). Livedo reticularis occurs also with polyarteritis nodosa, systemic lupus erythematosus, and central thrombocythemia, any one of which may be accompanied by cerebrovascular accidents (Bruyn et al., 1987).
Vasculitis due to ADA2 deficiency
MedGen UID:
854497
Concept ID:
C3887654
Disease or Syndrome
Adenosine deaminase 2 deficiency (DADA2) is a complex systemic autoinflammatory disorder in which vasculopathy/vasculitis, dysregulated immune function, and/or hematologic abnormalities may predominate. Inflammatory features include intermittent fevers, rash (often livedo racemosa/reticularis), and musculoskeletal involvement (myalgia/arthralgia, arthritis, myositis). Vasculitis, which usually begins before age ten years, may manifest as early-onset ischemic (lacunar) and/or hemorrhagic strokes, or as cutaneous or systemic polyarteritis nodosa. Hypertension and hepatosplenomegaly are often found. More severe involvement may lead to progressive central neurologic deficits (dysarthria, ataxia, cranial nerve palsies, cognitive impairment) or to ischemic injury to the kidney, intestine, and/or digits. Dysregulation of immune function can lead to immunodeficiency or autoimmunity of varying severity; lymphadenopathy may be present and some affected individuals have had lymphoproliferative disease. Hematologic disorders may begin early in life or in late adulthood, and can include lymphopenia, neutropenia, pure red cell aplasia, thrombocytopenia, or pancytopenia. Of note, both interfamilial and intrafamilial phenotypic variability (e.g., in age of onset, frequency and severity of manifestations) can be observed; also, individuals with biallelic ADA2 pathogenic variants may remain asymptomatic until adulthood or may never develop clinical manifestations of DADA2.

Professional guidelines

PubMed

Mitri F, Enk A, Bersano A, Kraemer M
Eur J Neurol 2020 Oct;27(10):1832-1843. Epub 2020 Jul 2 doi: 10.1111/ene.14390. PMID: 32526049
Kerk N, Goerge T
J Dtsch Dermatol Ges 2013 May;11(5):407-10. Epub 2013 Feb 26 doi: 10.1111/ddg.12064. PMID: 23437985
Kraemer M, Linden D, Berlit P
J Neurol 2005 Oct;252(10):1155-66. Epub 2005 Aug 26 doi: 10.1007/s00415-005-0967-9. PMID: 16133722

Recent clinical studies

Therapy

Chakraborty U, Banerjee S, Chandra A, Sil A
BMJ Case Rep 2024 Jan 12;17(1) doi: 10.1136/bcr-2023-257492. PMID: 38216170Free PMC Article
Ordi-Ros J, Sáez-Comet L, Pérez-Conesa M, Vidal X, Riera-Mestre A, Castro-Salomó A, Cuquet-Pedragosa J, Ortiz-Santamaria V, Mauri-Plana M, Solé C, Cortés-Hernández J
Ann Intern Med 2019 Nov 19;171(10):685-694. Epub 2019 Oct 15 doi: 10.7326/M19-0291. PMID: 31610549
Samanta D, Cobb S, Arya K
J Stroke Cerebrovasc Dis 2019 Aug;28(8):2098-2108. Epub 2019 May 31 doi: 10.1016/j.jstrokecerebrovasdis.2019.05.013. PMID: 31160219
Sebaratnam DF, Agar N
Med J Aust 2017 Oct 16;207(8):327. doi: 10.5694/mja16.01355. PMID: 29020898
Criado PR, Alavi A, Valente NY, Sotto MN
Int J Low Extrem Wounds 2016 Mar;15(1):78-81. Epub 2015 Sep 3 doi: 10.1177/1534734615603566. PMID: 26338517

Prognosis

Kolivras A, Thompson C
Clin Dermatol 2021 Mar-Apr;39(2):278-282. Epub 2020 Oct 16 doi: 10.1016/j.clindermatol.2020.10.011. PMID: 34272022
Gottlieb M, Long B
Am J Emerg Med 2020 Sep;38(9):1715-1721. Epub 2020 Jun 6 doi: 10.1016/j.ajem.2020.06.011. PMID: 32731141Free PMC Article
Samanta D, Cobb S, Arya K
J Stroke Cerebrovasc Dis 2019 Aug;28(8):2098-2108. Epub 2019 May 31 doi: 10.1016/j.jstrokecerebrovasdis.2019.05.013. PMID: 31160219
Sunderkötter C, Sindrilaru A
Eur J Dermatol 2006 Mar-Apr;16(2):114-24. PMID: 16581560
Kraemer M, Linden D, Berlit P
J Neurol 2005 Oct;252(10):1155-66. Epub 2005 Aug 26 doi: 10.1007/s00415-005-0967-9. PMID: 16133722

Clinical prediction guides

Karppinen JJ, Kallio M, Lappalainen K, Lagus H, Matikainen N, Isoherranen K
J Wound Care 2023 Dec 2;32(12):797-804. doi: 10.12968/jowc.2023.32.12.797. PMID: 38060417
Kolivras A, Thompson C, Pastushenko I, Mathieu M, Bruderer P, de Vicq M, Feoli F, Harag S, Meiers I, Olemans C, Sass U, Dehavay F, Fakih A, Lam-Hoai XL, Marneffe A, Van De Borne L, Vandersleyen V, Richert B
J Cutan Pathol 2022 Jan;49(1):17-28. Epub 2021 Aug 9 doi: 10.1111/cup.14099. PMID: 34272741Free PMC Article
Grangeon L, Guey S, Schwitalla JC, Bergametti F, Arnould M, Corpechot M, Hadjadj J, Riant F, Aloui C, Drunat S, Vidaud D, Tournier-Lasserve E, Kraemer M
Stroke 2019 Apr;50(4):789-796. doi: 10.1161/STROKEAHA.118.023972. PMID: 30908154
Chasset F, Francès C
Clin Rev Allergy Immunol 2017 Dec;53(3):452-468. doi: 10.1007/s12016-017-8612-9. PMID: 28547523
Thompson AK, Peters MS, El-Azhary RA, Gibson LE, Chang MB, Griffin JR, Davis MD, McEvoy MT, Camilleri MJ, Bridges AG
J Am Acad Dermatol 2015 Oct;73(4):666-71. Epub 2015 Aug 12 doi: 10.1016/j.jaad.2015.07.014. PMID: 26277212

Recent systematic reviews

Loiseau P, Foret T, DeFilippis EM, Risse J, Etienne AD, Dufrost V, Moulinet T, Erkan D, Devilliers H, Wahl D, Zuily S
Lupus 2022 Nov;31(13):1595-1605. Epub 2022 Sep 15 doi: 10.1177/09612033221126852. PMID: 36112747

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