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CAUSES OF LIVEDOID VASCULITIS

A number of conditions may cause the appearance of livedo reticularis:

  • Cutis marmorata telangiectatica congenita, a rare congenital condition
  • Sneddon syndrome - association of livedoid vasculitis and systemic vascular disorders, such as strokes, due to underlying genetic cause[9]
  • Idiopathic livedo reticularis - the most common form of livedo reticularis, completely benign condition of unknown cause affecting mostly young women during the winter:[10] It is a lacy purple appearance of skin in extremities due to sluggish venous blood flow. It may be mild, but ulceration may occur later in the summer.[11]
  • Secondary livedo reticularis:
    • Vasculitis autoimmune conditions:
    • Drug-related:
    • Obstruction of capillaries:
    • Ehlers-Danlos syndrome—connective tissue disorder, often with many secondary conditions, may be present in all types
      • Beta IFN treatment in i.e. multiple sclerosis[18][19][20]
      • Pheochromocytoma[21]
      • Livedoid vasculopathy and its association with factor V Leiden mutation[22]
      • FILS syndrome (polymerase ε1 mutation in a human syndrome with facial dysmorphism, immunodeficiency, livedo, and short stature)[23]
      • Primary hyperoxaluria, oxalosis (oxalate vasculopathy)[24][25][26][27][28]
      • Cytomegalovirus infection (very rare clinical form, presenting with persistent fever and livedo reticularis on the extremities and cutaneous necrotizing vasculitis of the toes)[29]
      • Livedo reticularis associated with rasagiline[30]
      • Generalized livedo reticularis induced by silicone implants for soft tissue augmentation[31]
      • As a rare skin finding in children with Down syndrome[32][33]
      • Idiopathic livedo reticularis with polyclonal IgM hypergammopathy[34]
      • CO2 angiography (rare, reported case)[35]
      • A less common skin lesion of Churg-Strauss syndrome[36]
      • Erythema nodosum-like cutaneous lesions of sarcoidosis showing livedoid changes in a patient with sarcoidosis and Sjögren's syndrome[37]
      • Livedo vasculopathy associated with IgM antiphosphatidylserine-prothrombin complex antibody[38]
      • Livedo vasculopathy associated with plasminogen activator inhibitor-1 promoter homozygosity and prothrombin G20210A heterozygosity[38]
      • As a first sign of metastatic breast carcinoma (very rare)[39]
      • Livedo reticularis associated with renal cell carcinoma (rare)[40]
      • Methylphenidate and dextroamphetamine-induced peripheral vasculopathy[41]
      • Gefitinib[42]
      • Buerger's disease (as an initial symptom)[43]
      • As a rare manifestation of Graves hyperthyroidism[44]
      • Associated with pernicious anaemia[45]
      • Moyamoya disease (a rare, chronic cerebrovascular occlusive disease of unknown etiology, characterized by progressive stenosis of the arteries of the circle of Willis leading to an abnormal capillary network and resultant ischemic strokes or cerebral hemorrhages)[46]
      • Associated with the use of a midline catheter[47]
      • Familial primary cryofibrinogenemia.[48]




 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 









Primary treatment option
Atropine
❑ Administer a first dose 0.5 mg IV bolus
❑ Repeat every 3-5 minutes
❑ Administer a maximum dose of 3 mg
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Secondary treatment options
If atropine ineffective:
❑ Administer dopamine infusion (2-10 mcg/kg/min)
OR
❑ Administer epinephrine infusion (2-10 mcg/min)
OR
❑ Proceed with transcutaneous pacing
 
 
 
 
 
 
 
 
 
 
 
 
 
 
❑ Consult a cardiologist
❑ Consider transvenous pacing
 
 
 
 
 
 






References

  1. Sneddon I (1965). "Cerebro-Vascular Lesions And Livedo Reticularis". Br J Dermatol. 77: 180–5. PMID 14278790. Unknown parameter |month= ignored (help)
  2. Gibbs M, English J, Zirwas M (2005). "Livedo reticularis: an update". J Am Acad Dermatol. 52 (6): 1009–19. PMID 15928620.
  3. Feldaker M, Hines E, Kierland R (1955). "Livedo reticularis with summer ulcerations". AMA Arch Derm. 72 (1): 31–42. PMID 14387292.
  4. Golden R (1963). "Livedo reticularis in systemic lupus erythematosus". Arch Dermatol. 87: 299–301. PMID 13948706. Unknown parameter |month= ignored (help)
  5. Sigmund W, Shelley W (1954). "Cutaneous manifestations of acute pancreatitis, with special reference to livedo reticularis". N Engl J Med. 251 (21): 851–3. PMID 13214346.
  6. Langhof H, Braun G, Matzkowski H (1957). "[Livedo reticularis due to cold gelation of the blood by gamma-plasmacytoma.]". Arch Klin Exp Dermatol. 205 (4): 343–50. PMID 13522017.
  7. Kazmier F, Sheps S, Bernatz P, Sayre G (1966). "Livedo reticularis and digital infarcts: a syndrome due to cholesterol emboli arising from atheromatous abdominal aortic aneurysms". Vasc Dis. 3 (1): 12–24. PMID 5903590.
  8. Stewart W, Lauret P, Testart J, Thomine E, Boulliê M, Leroy D (1977). "[Cutaneous cholesterol emboli (author's transl)]". Ann Dermatol Venereol. 104 (1): 5–8. PMID 843026.
  9. Sneddon, I. B. (April 1965). "Cerebro-Vascular Lesions And Livedo Reticularis". British Journal of Dermatology. 77 (4): 180–5. doi:10.1111/j.1365-2133.1965.tb14628.x. PMID 14278790. |first2= missing |last2= in Authors list (help)
  10. Gibbs, Mark B.; English, Joseph C.; Zirwas, Matthew J. (2005). "Livedo reticularis: an update". J Am Acad Dermatol. 52 (6): 1009–19. doi:10.1016/j.jaad.2004.11.051. PMID 15928620.
  11. Feldaker M, Hines E, Kierland R (1955). "Livedo reticularis with summer ulcerations". AMA Arch Derm. 72 (1): 31–42. doi:10.1001/archderm.1955.03730310033007. PMID 14387292.
  12. Golden R (March 1963). "Livedo reticularis in systemic lupus erythematosus". Arch Dermatol. 87: 299–301. doi:10.1001/archderm.1963.01590150015002. PMID 13948706.
  13. Sigmund W, Shelley W (1954). "Cutaneous manifestations of acute pancreatitis, with special reference to livedo reticularis". N Engl J Med. 251 (21): 851–3. doi:10.1056/NEJM195411182512104. PMID 13214346.
  14. Gould, Jennifer W.; Helms, Stephen E.; Schulz, Susan M.; Stevens, Seth R. (1998). "Relapsing livedo reticularis in the setting of chronic pancreatitis". Journal of the American Academy of Dermatology. 39 (6): 1035–1036. doi:10.1016/S0190-9622(98)70290-7. PMID 9843029.
  15. Langhof H, Braun G, Matzkowski H (1957). "Livedo reticularis durch Kältegelierung des Blutes bei γ-Plasmocytom". Archiv für klinische und experimentelle Dermatologie (in Deutsch). 205 (4): 343–50. doi:10.1007/BF00693523. PMID 13522017. Unknown parameter |trans_title= ignored (help)
  16. Kazmier F, Sheps S, Bernatz P, Sayre G (1966). "Livedo reticularis and digital infarcts: a syndrome due to cholesterol emboli arising from atheromatous abdominal aortic aneurysms". Vasc Dis. 3 (1): 12–24. PMID 5903590.
  17. Stewart W, Lauret P, Testart J, Thomine E, Boulliê M, Leroy D (1977). "Les manifestations cutanées des emoblies de critaux de cholestérol". Ann Dermatol Venereol (in français). 104 (1): 5–8. PMID 843026. Unknown parameter |trans_title= ignored (help)
  18. Rot, Uroš; Ledinek, Alenka Horvat (December 2013). "Interferons beta have vasoconstrictive and procoagulant effects: a woman who developed livedo reticularis and Raynaud phenomenon in association with interferon beta treatment for multiple sclerosis". Clinical Neurology and Neurosurgery. 115 (Suppl 1): S79–81. doi:10.1016/j.clineuro.2013.09.027. PMID 24321162.
  19. Fox, Michelle; Tahan, Steven; Kim, Caroline C. (2012). "Livedo Reticularis: A Side Effect of Interferon Therapy in a Pediatric Patient with Melanoma". Pediatric Dermatology. 29 (3): 333–5. doi:10.1111/j.1525-1470.2011.01426.x. PMID 21575046.
  20. Cruz, Boris Afonso; de Queiroz, Eustáquio; Nunes, Simone Vilela; Cruz Filho, Achiles; Campos, Gilberto Belisario; de Carvalho Monteiro, Ernesto Lentz; Crivellari, Humberto (2000). "Fênomeno de Raynaud grave associado a terapia com interferon-beta para esclerose múltipla: relato de caso". Arquivos de Neuro-Psiquiatria (in Portuguese). 58 (2B): 556–9. doi:10.1590/S0004-282X2000000300025. PMID 10920422. Unknown parameter |trans_title= ignored (help)
  21. Buckley, Sarah A.; Lessing, Juan N.; Mark, Nicholas M. (2013). "Livedo Reticularis in a Patient with Pheochromocytoma Resolving After Adrenalectomy". The Journal of Clinical Endocrinology & Metabolism. 98 (2): 439–40. doi:10.1210/jc.2012-2842.
  22. "Livedoid vasculopathy and its association with factor V Leiden mutation" (PDF). PMID 23268168.
  23. Pachlopnik Schmid, Jana; Lemoine; Nehme, Nadine; Cormier-Daire, Valéry; Revy, Patrick; Debeurme, Franck; Debré, Marianne; Nitschke, Patrick; Bole-Feysot, Christine; Legeai-Mallet, Laurence; Lim, Annick; de Villartay, Jean-Pierre; Picard, Capucine; Durandy, Anne; Fischer, Alain; de Saint Basile, Geneviève (2012). "Polymerase ε1 mutation in a human syndrome with facial dysmorphism, immunodeficiency, livedo, and short stature ('FILS syndrome')". Journal of Experimental Medicine. 209 (13): 2323–30. doi:10.1084/jem.20121303. PMC 3526359. PMID 23230001.
  24. Jorquera-Barquero, E.; Súarez-Marrero, M.C.; Fernández Girón, F.; Borrero Martín, J.J. (2013). "Oxalosis y livedo reticularis". Actas Dermo-Sifiliográficas (in Spanish). 104 (9): 815–8. doi:10.1016/j.ad.2012.04.019. PMID 23103120. Unknown parameter |trans_title= ignored (help)
  25. "Primary hyperoxaluria in a 27-year-old woman". PMID 14512927.
  26. "Primary hyperoxaluria: report of a patient with livedo reticularis and digital infarcts". PMID 11807460.
  27. "Livedo reticularis, ulcers, and peripheral gangrene: cutaneous manifestations of primary hyperoxaluria". PMID 11030785.
  28. "Steroid-responsive pleuropericarditis and livedo reticularis in an unusual case of adult-onset primary hyperoxaluria". PMID 10196036.
  29. Arslan, Ferhat; Batirel, Ayse; Mert, Ali; Ozer, Serdar (2012). "Cytomegalovirus (CMV)-related cutaneous necrotizing vasculitis: Case report and literature review". The Brazilian Journal of Infectious Diseases. 16 (5): 482–5. doi:10.1016/j.bjid.2012.08.002. PMID 22975173.
  30. Strowd, Lindsay C.; Lee, Andrew D.; Yosipovitch, Gil (June 2012). "Livedo Reticularis Associated With Rasagiline (Azilect)". Journal of Drugs in Dermatology. 11 (6): 764–5. PMID 22648227.
  31. Camacho, Diana; Machan, Shalma; Pilesanski, Ursula; Revelles, Juan Maria; Martín, Lucia; Requena, Luis (2012). "Generalized Livedo Reticularis Induced by Silicone Implants for Soft Tissue Augmentation". The American Journal of Dermatopathology. 34 (2): 203–7. doi:10.1097/DAD.0b013e31821cb3c5. PMID 22441370.
  32. "Mucocutaneous disorders in children with down syndrome: case-controlled study". PMID 22303799.
  33. "Mucocutaneous findings in 100 children with Down syndrome". PMID 17542890.
  34. "John Libbey Eurotext : Éditions médicales et scientifiques France : revues, médicales, scientifiques, médecine, santé, livres". Jle.com. Retrieved 2014-03-19.[dead link]
  35. Johnson, Philip L.; Neperud, Julie; Arnold, Jill; Thomas, James (2011). "Livedo Reticularis and Bowel Ischemia after Carbon Dioxide Arteriography in a Patient with CREST Syndrome". Journal of Vascular and Interventional Radiology. 22 (3): 395. doi:10.1016/j.jvir.2010.11.012. PMID 21277800.
  36. Bosco, Laura; Peroni, Anna; Schena, Donatella; Colato, Chiara; Girolomoni, Giampiero (2010). "Cutaneous manifestations of Churg–Strauss syndrome: Report of two cases and review of the literature". Clinical Rheumatology. 30 (4): 573–80. doi:10.1007/s10067-010-1593-1. PMID 20949297.
  37. "John Libbey Eurotext : Éditions médicales et scientifiques France : revues, médicales, scientifiques, médecine, santé, livres". Jle.com. Retrieved 2014-03-19.[dead link]
  38. 38.0 38.1 Tabata, N; Oonami, K; Ishibashi, M; Yamazaki, M (2010). "Livedo Vasculopathy Associated with IgM Anti-phosphatidylserine-prothrombin Complex Antibody". Acta Dermato Venereologica. 90 (3): 313–4. doi:10.2340/00015555-0835. PMID 20526560.
  39. Gambichler, T.; Baier, P.; Altmeyer, P. (2009). "Generalized livedo reticularis as the first sign of metastatic breast carcinoma". Clinical and Experimental Dermatology. 34 (2): 253. doi:10.1111/j.1365-2230.2008.02801.x. PMID 19120398.
  40. Erel, Arzu; Ozsoy, Esra; University, Gazi (2001). "Livedo reticularis associated with renal cell carcinoma". International Journal of Dermatology. 40 (4): 299. doi:10.1046/j.1365-4362.2001.00895.x. PMID 11454094.
  41. Syed, Reema H.; Moore, Terry L. (2008). "Methylphenidate and Dextroamphetamine-Induced Peripheral Vasculopathy". JCR: Journal of Clinical Rheumatology. 14: 30. doi:10.1097/RHU.0b013e3181639aaa.
  42. Blume, Jonathan E.; Miller, Craig C. (2007). "Livedo reticularis with retiform purpura associated with gefitinib (Iressa®)". International Journal of Dermatology. 46 (12): 1307. doi:10.1111/j.1365-4632.2007.03319.x. PMID 18173531.
  43. Takanashi, Tetsuo; Horigome, Reiko; Okuda, Yasuaki; Nose, Masato; Matsuda, Masayuki; Ikeda, Shu-Ichi (2007). "Buerger's Disease Manifesting Nodular Erythema with Livedo Reticularis". Internal Medicine. 46 (21): 1815. doi:10.2169/internalmedicine.46.0143. PMID 17978541.
  44. "Livedo reticularis: a rare manifestation of Graves hyperthyroidism associated with anticardiolipin antibodies". PMID 15255431.
  45. Bandyopadhyay, D. (2003). "Celecoxib-induced fixed drug eruption". Clinical and Experimental Dermatology. 28 (4): 452. doi:10.1046/j.1365-2230.2003.01285_5.x.
  46. Richards, Kristen A.; Paller, Amy S. (2003). "Livedo Reticularis in a Child with Moyamoya Disease". Pediatric Dermatology. 20 (2): 124. doi:10.1046/j.1525-1470.2003.20205.x. PMID 12657007.
  47. "Livedo reticularis associated with the use of a midline catheter". PMID 12578157.
  48. "Familial primary cryofibrinogenemia". PMID 10188150.

Overview

Causes

Life Threatening Causes

Common Causes

Diagnosis

Focused Initial Rapid Evaluation

Complete Evaluation

 
 
 
 
 
 
 
 
A01
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
B01
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
C01
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
D01
 
 
 
 
 
 
 
 
 
 
 
D02
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
E01
 
 
 
 
 
 
 
 
 
 
 
E02
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
F01
 
 
 
 
 
 
 
 
 
 
 
 


Treatment

Do's

Don'ts

References

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