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Livedo reticularis

MedGen UID:
43223
Concept ID:
C0085642
Disease or Syndrome
Synonym: Livedo Reticularis
SNOMED CT: Livedo reticularis (238772004); Livedo racemosa (238772004)
 
HPO: HP:0033505
Monarch Initiative: MONDO:0044037

Definition

Livedo reticularis is characterized by the presence of a bluish purple, mottled or netlike pattern in unbroken circles on the skin. Exposure to cold environments usually intensifies the vascular pattern. Presumably, the condition results from slow or stagnant blood flow, vessel-wall pathology, and decreased oxygen tension. [from HPO]

Term Hierarchy

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

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).
Aortic aneurysm, familial thoracic 4
MedGen UID:
338704
Concept ID:
C1851504
Disease or Syndrome
Any familial thoracic aortic aneurysm and aortic dissection in which the cause of the disease is a mutation in the MYH11 gene.
Aortic aneurysm, familial thoracic 6
MedGen UID:
435866
Concept ID:
C2673186
Disease or Syndrome
Any familial thoracic aortic aneurysm and aortic dissection in which the cause of the disease is a mutation in the ACTA2 gene.
Deafness-encephaloneuropathy-obesity-valvulopathy syndrome
MedGen UID:
766268
Concept ID:
C3553354
Disease or Syndrome
Primary coenzyme Q10 (CoQ10) deficiency is usually associated with multisystem involvement, including neurologic manifestations such as fatal neonatal encephalopathy with hypotonia; a late-onset slowly progressive multiple-system atrophy-like phenotype (neurodegeneration with autonomic failure and various combinations of parkinsonism and cerebellar ataxia, and pyramidal dysfunction); and dystonia, spasticity, seizures, and intellectual disability. Steroid-resistant nephrotic syndrome (SRNS), the hallmark renal manifestation, is often the initial manifestation either as isolated renal involvement that progresses to end-stage renal disease (ESRD), or associated with encephalopathy (seizures, stroke-like episodes, severe neurologic impairment) resulting in early death. Hypertrophic cardiomyopathy (HCM), retinopathy or optic atrophy, and sensorineural hearing loss can also be seen.
Moyamoya disease with early-onset achalasia
MedGen UID:
816733
Concept ID:
C3810403
Disease or Syndrome
Moyamoya disease-6 is a progressive vasculopathy characterized by occlusion of the terminal portion of the internal carotid arteries and its branches, and the formation of compensatory neovascularization and the moyamoya, or 'puff of smoke,' appearance of these vessels on angiogram. Affected individuals may present with ischemic strokes, intracerebral hemorrhage, or transient ischemic attacks. Patients with MYMY6 usually present early in life with achalasia. Hypertension and Raynaud phenomenon may be associated features (summary by Wallace et al., 2016; Herve et al., 2014). For a general phenotypic description and a discussion of genetic heterogeneity of moyamoya disease, see MYMY1 (252350).
STING-associated vasculopathy with onset in infancy
MedGen UID:
863159
Concept ID:
C4014722
Disease or Syndrome
STING-associated vasculopathy with onset in infancy is an autoinflammatory vasculopathy causing severe skin lesions, particularly affecting the face, ears, nose, and digits, and resulting in ulceration, eschar formation, necrosis, and, in some cases, amputation. Many patients have interstitial lung disease. Tissue biopsy and laboratory findings show a hyperinflammatory state, with evidence of increased beta-interferon (IFNB1; 147640) signaling (summary by Liu et al., 2014).
RECON progeroid syndrome
MedGen UID:
1841140
Concept ID:
C5830504
Disease or Syndrome
RECON progeroid syndrome (RECON) is a chromosomal instability disorder characterized by postnatal growth retardation, progeroid facial appearance, hypoplastic nose, prominent premaxilla, skin photosensitivity and xeroderma, muscle wasting with reduced subcutaneous fat, and slender elongated thumbs (Abu-Libdeh et al., 2022).

Professional guidelines

PubMed

Uthman I, Noureldine MHA, Ruiz-Irastorza G, Khamashta M
Ann Rheum Dis 2019 Feb;78(2):155-161. Epub 2018 Oct 3 doi: 10.1136/annrheumdis-2018-213846. PMID: 30282668
Meyts I, Aksentijevich I
J Clin Immunol 2018 Jul;38(5):569-578. Epub 2018 Jun 27 doi: 10.1007/s10875-018-0525-8. PMID: 29951947Free PMC Article
Sciascia S, Amigo MC, Roccatello D, Khamashta M
Nat Rev Rheumatol 2017 Sep;13(9):548-560. Epub 2017 Aug 3 doi: 10.1038/nrrheum.2017.124. PMID: 28769114

Recent clinical studies

Etiology

Genovese G, Moltrasio C, Berti E, Marzano AV
Dermatology 2021;237(1):1-12. Epub 2020 Nov 24 doi: 10.1159/000512932. PMID: 33232965Free PMC Article
Sammaritano LR
Best Pract Res Clin Rheumatol 2020 Feb;34(1):101463. Epub 2019 Dec 19 doi: 10.1016/j.berh.2019.101463. PMID: 31866276
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
Meyts I, Aksentijevich I
J Clin Immunol 2018 Jul;38(5):569-578. Epub 2018 Jun 27 doi: 10.1007/s10875-018-0525-8. PMID: 29951947Free PMC Article
Micieli R, Alavi A
JAMA Dermatol 2018 Feb 1;154(2):193-202. doi: 10.1001/jamadermatol.2017.4374. PMID: 29141075

Diagnosis

Kolopp-Sarda MN, Miossec P
Curr Opin Rheumatol 2021 Jan;33(1):1-7. doi: 10.1097/BOR.0000000000000757. PMID: 33186245
Sammaritano LR
Best Pract Res Clin Rheumatol 2020 Feb;34(1):101463. Epub 2019 Dec 19 doi: 10.1016/j.berh.2019.101463. PMID: 31866276
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
Meyts I, Aksentijevich I
J Clin Immunol 2018 Jul;38(5):569-578. Epub 2018 Jun 27 doi: 10.1007/s10875-018-0525-8. PMID: 29951947Free PMC Article
Sciascia S, Amigo MC, Roccatello D, Khamashta M
Nat Rev Rheumatol 2017 Sep;13(9):548-560. Epub 2017 Aug 3 doi: 10.1038/nrrheum.2017.124. PMID: 28769114

Therapy

Nakashima C, Kato M, Otsuka A
J Dermatol 2023 Mar;50(3):280-289. Epub 2023 Jan 13 doi: 10.1111/1346-8138.16651. PMID: 36636825
Niemann N, Billnitzer A, Jankovic J
Parkinsonism Relat Disord 2021 Jan;82:61-76. Epub 2020 Nov 20 doi: 10.1016/j.parkreldis.2020.11.017. PMID: 33248395
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
Uthman I, Noureldine MHA, Ruiz-Irastorza G, Khamashta M
Ann Rheum Dis 2019 Feb;78(2):155-161. Epub 2018 Oct 3 doi: 10.1136/annrheumdis-2018-213846. PMID: 30282668
Micieli R, Alavi A
JAMA Dermatol 2018 Feb 1;154(2):193-202. doi: 10.1001/jamadermatol.2017.4374. PMID: 29141075

Prognosis

Casanegra AI, Shepherd RF
Cardiol Clin 2021 Nov;39(4):583-599. doi: 10.1016/j.ccl.2021.06.010. PMID: 34686269
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
Micieli R, Alavi A
JAMA Dermatol 2018 Feb 1;154(2):193-202. doi: 10.1001/jamadermatol.2017.4374. PMID: 29141075
Sangle SR, D'Cruz DP
Isr Med Assoc J 2015 Feb;17(2):104-7. PMID: 26223086
Marzano AV, Balice Y, Tavecchio S, Desimine C, Colombo A, Berti E
G Ital Dermatol Venereol 2015 Apr;150(2):193-202. Epub 2015 Mar 19 PMID: 25791629

Clinical prediction guides

Nakashima C, Kato M, Otsuka A
J Dermatol 2023 Mar;50(3):280-289. Epub 2023 Jan 13 doi: 10.1111/1346-8138.16651. PMID: 36636825
Santiago MB, Melo BS
Curr Rheumatol Rev 2022;18(3):186-194. doi: 10.2174/1573397118666220325110737. PMID: 35339184
Micieli R, Alavi A
JAMA Dermatol 2018 Feb 1;154(2):193-202. doi: 10.1001/jamadermatol.2017.4374. PMID: 29141075
Volpi S, Picco P, Caorsi R, Candotti F, Gattorno M
Pediatr Rheumatol Online J 2016 Jun 4;14(1):35. doi: 10.1186/s12969-016-0094-4. PMID: 27260006Free PMC Article
Cervera R, Serrano R, Pons-Estel GJ, Ceberio-Hualde L, Shoenfeld Y, de Ramón E, Buonaiuto V, Jacobsen S, Zeher MM, Tarr T, Tincani A, Taglietti M, Theodossiades G, Nomikou E, Galeazzi M, Bellisai F, Meroni PL, Derksen RH, de Groot PG, Baleva M, Mosca M, Bombardieri S, Houssiau F, Gris JC, Quéré I, Hachulla E, Vasconcelos C, Fernández-Nebro A, Haro M, Amoura Z, Miyara M, Tektonidou M, Espinosa G, Bertolaccini ML, Khamashta MA; Euro-Phospholipid Project Group (European Forum on Antiphospholipid Antibodies)
Ann Rheum Dis 2015 Jun;74(6):1011-8. Epub 2014 Jan 24 doi: 10.1136/annrheumdis-2013-204838. PMID: 24464962

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
Kasap Cuceoglu M, Sener S, Batu ED, Kaya Akca U, Demir S, Sag E, Atalay E, Balık Z, Basaran O, Bilginer Y, Ozen S
Semin Arthritis Rheum 2021 Jun;51(3):559-564. Epub 2021 Apr 19 doi: 10.1016/j.semarthrit.2021.04.009. PMID: 33901990
Micieli R, Alavi A
JAMA Dermatol 2018 Feb 1;154(2):193-202. doi: 10.1001/jamadermatol.2017.4374. PMID: 29141075
Medlin JL, Hansen KE, Fitz SR, Bartels CM
Semin Arthritis Rheum 2016 Jun;45(6):691-7. Epub 2016 Jan 21 doi: 10.1016/j.semarthrit.2016.01.004. PMID: 26972993Free PMC Article
Schlienger RG, Bircher AJ, Meier CR
Dermatology 2000;200(3):223-31. doi: 10.1159/000018387. PMID: 10828631

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