Skip to main content

Advertisement

Log in

Eosinophilic Vasculitis: Time for Recognition of a New Entity?

  • Case Report
  • Published:
Indian Journal of Hematology and Blood Transfusion Aims and scope Submit manuscript

Abstract

Hypereosinophilia is part of a group of complex disorders with multisystem involvement. A 23 year old male was admitted to our centre with bilateral popliteal artery and venous thrombosis and impending gangrene of the left forefoot along with deep venous thrombosis of the right lower extremity. Investigations revealed marked peripheral blood eosinophilia (27,669/μL). Bone marrow showed increased eosinophils & eosinophil precursors and no evidence of a clonal disorder. Skin biopsy from the ulcerated lesions showed small vessel vasculitis with intense eosinophilic infiltration. Investigations to look for secondary causes of hypereosinophilia in the form of Antinuclear Antibody, P-Anti Neutrophil Cytoplasmic Antibody (ANCA) and C-ANCA and FIP1L1-PDGFRA, Bcr-Abl and JAK2V617F mutations were negative. The arterial and venous thrombosis and cutaneous vasculitis were linked to the presence of hypereosinophilic syndrome. The patient’s illness responded to high dose corticosteroids leading to complete resolution of symptoms. We reviewed the literature on the lesser known entity of eosinophilic vasculitis and its association with thrombosis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4

References

  1. Chusid MJ, Dale DC, West BC, Wolff SM (1975) The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore) 54(1):1–27 Epub 1975/01/01

    Article  CAS  Google Scholar 

  2. Valent P, Klion AD, Horny HP, Roufosse F, Gotlib J, Weller PF et al (2012) Contemporary consensus proposal on criteria and classification of eosinophilic disorders and related syndromes. J Allergy Clin Immunol 130(3):607-12 e9 Epub 2012/03/31

    Google Scholar 

  3. Chen KR, Pittelkow MR, Su D, Gleich J, Newman W, Leiferman KM (1994) Recurrent cutaneous necrotizing eosinophilic vasculitis. a novel eosinophil-mediated syndrome. Arch Dermatol 130(9):1159–1166 Epub 1994/09/01

    Article  PubMed  CAS  Google Scholar 

  4. Liao YH, Su YW, Tsay W, Chiu HC (2005) Association of cutaneous necrotizing eosinophilic vasculitis and deep vein thrombosis in hypereosinophilic syndrome. Arch Dermatol 141(8):1051–1053 Epub 2005/08/17

    PubMed  Google Scholar 

  5. Moosbauer C, Morgenstern E, Cuvelier SL, Manukyan D, Bidzhekov K, Albrecht S et al (2007) Eosinophils are a major intravascular location for tissue factor storage and exposure. Blood 109(3):995–1002 Epub 2006/09/28

    Article  PubMed  CAS  Google Scholar 

  6. Venge P, Dahl R, Hallgren R (1979) Enhancement of factor XII dependent reactions by eosinophil cationic protein. Thromb Res 14(4–5):641–649 Epub 1979/01/01

    Article  PubMed  CAS  Google Scholar 

  7. Fan TC, Fang SL, Hwang CS, Hsu CY, Lu XA, Hung SC et al (2008) Characterization of molecular interactions between eosinophil cationic protein and heparin. J Biol Chem 283(37):25468–25474 Epub 2008/07/03

    Article  PubMed  CAS  Google Scholar 

  8. Mukai HY, Ninomiya H, Ohtani K, Nagasawa T, Abe T (1995) Major basic protein binding to thrombomodulin potentially contributes to the thrombosis in patients with eosinophilia. Br J Haematol 90(4):892–899 Epub 1995/08/01

    Article  PubMed  CAS  Google Scholar 

  9. Gleich GJ, Frigas E, Loegering DA, Wassom DL, Steinmuller D (1979) Cytotoxic properties of the eosinophil major basic protein. J Immunol 123(6):2925–2927 Epub 1979/12/01

    PubMed  CAS  Google Scholar 

  10. Abdulwahab A, Almoallim H, Khan N (2007) Isolated eosinophilic mesenteric vasculitis with extensive thrombosis and splenic infarction in a 13-year-old boy. Clin Rheumatol 26(2):254–257 Epub 2005/12/08

    Article  PubMed  Google Scholar 

  11. Cecchi PC, Caramaschi P, Pinna G, Schwarz A, Bricolo A (2006) Haemorrhagic stroke and vasculitic-like cerebral angiography in a patient with eosinophilic fasciitis. case report. J Neurosurg Sci 50(4):119–122 Epub 2007/02/08

    PubMed  CAS  Google Scholar 

  12. Song JK, Jung SS, Kang SW (2008) Two cases of eosinophilic vasculitis with thrombosis. Rheumatol Int 28(4):371–374 Epub 2007/08/19

    Article  PubMed  Google Scholar 

  13. Ko JH, Lin JW, Hui RC (2011) Acute pulmonary embolism in a patient with hypereosinophilia and psoriasis. Chang Gung Med J 34(6 Suppl):17–23 Epub 2012/04/18

    PubMed  Google Scholar 

  14. Antoniu SA (2006) Imatinib mesylate for the treatment of hypereosinophilic syndromes. Curr Opin Investig Drugs 7(11):980–984 Epub 2006/11/23

    PubMed  CAS  Google Scholar 

  15. Butterfield JH, Weiler CR (2012) Treatment of hypereosinophilic syndromes–the first 100 years. Semin Hematol 49(2):182–191 Epub 2012/03/28

    Article  PubMed  CAS  Google Scholar 

  16. Roufosse FE, Kahn JE, Gleich GJ, Schwartz LB, Singh AD, Rosenwasser LJ et al (2013) Long-term safety of mepolizumab for the treatment of hypereosinophilic syndromes. J Allergy Clin Immunol 131(2):461-7 e1-5 Epub 2012/10/09

    Google Scholar 

  17. Varma N, Varma S, Marwaha N, Dash S (1994) Hypereosinophilic syndrome: the spectrum of clinical, haematological and morphological features. J Assoc Physicians India 42(3):242–244 Epub 1994/03/01

    PubMed  CAS  Google Scholar 

  18. Ishii T, Koide O, Hosoda Y, Takahashi R (1977) Hypereosinophilic multiple thrombosis. a proposal of a new designation of disseminated eosinophilic “collagen disease”. Angiology 28(6):361–375 Epub 1977/06/01

    Article  PubMed  CAS  Google Scholar 

  19. Parker CJ (1988) Hypereosinophilic syndrome with cutaneous blisters and bowel necrosis. Australas J Dermatol 29(2):103–106 Epub 1988/01/01

    Article  PubMed  CAS  Google Scholar 

  20. Schulman H, Hertzog L, Zirkin H, Hertzanu Y (1999) Cerebral sinovenous thrombosis in the idiopathic hypereosinophilic syndrome in childhood. Pediatr Radiol 29(8):595–597 Epub 1999/07/23

    Article  PubMed  CAS  Google Scholar 

  21. Jang KA, Lim YS, Choi JH, Sung KJ, Moon KC, Koh JK (2000) Hypereosinophilic syndrome presenting as cutaneous necrotizing eosinophilic vasculitis and Raynaud’s phenomenon complicated by digital gangrene. Br J Dermatol 143(3):641–644 Epub 2000/09/06

    Article  PubMed  CAS  Google Scholar 

  22. Narayan S, Ezughah F, Standen GR, Pawade J, Kennedy CT (2003) Idiopathic hypereosinophilic syndrome associated with cutaneous infarction and deep venous thrombosis. Br J Dermatol 148(4):817–820 Epub 2003/05/20

    Article  PubMed  CAS  Google Scholar 

  23. Kim SH, Kim TB, Yun YS, Shin JI, Oh IY, Sir JJ et al (2005) Hypereosinophilia presenting as eosinophilic vasculitis and multiple peripheral artery occlusions without organ involvement. J Korean Med Sci 20(4):677–679 Epub 2005/08/16

    Article  PubMed  PubMed Central  Google Scholar 

  24. Aslan V, Entok E (2006) Factor V Leiden mutation and deep venous thrombosis in a patient with hypereosinophilic syndrome. Saudi Med J 27(9):1418–1420 Epub 2006/09/05

    PubMed  Google Scholar 

  25. Nakajima H, Nakamura T (2009) Thrombotic eosinophilic vasculitis. Clin Exp Dermatol 34(5):e69–e71 Epub 2009/05/15

    Article  PubMed  CAS  Google Scholar 

  26. Jandus P, Bianda N, Alerci M, Gallino A, Marone C (2010) Eosinophilic vasculitis: an inhabitual and resistant manifestation of a vasculitis. Vasa 39(4):344–348 Epub 2010/11/26

    Article  PubMed  CAS  Google Scholar 

Download references

Conflict of interest

The authors declare that they have no conflict of interest.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Arjun Datt Law.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Law, A.D., Varma, S., Varma, N. et al. Eosinophilic Vasculitis: Time for Recognition of a New Entity?. Indian J Hematol Blood Transfus 30 (Suppl 1), 325–330 (2014). https://doi.org/10.1007/s12288-014-0384-2

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12288-014-0384-2

Keywords

Navigation