Allergy Asthma Immunol Res.  2014 Mar;6(2):179-182. 10.4168/aair.2014.6.2.179.

A Case of Eosinophilic Fasciitis Presenting as Pitting Edema of the Lower Extremities

Affiliations
  • 1Department of Pediatrics and Institute of Allergy, Yonsei University College of Medicine, Seoul, Korea. kekim@yuhs.ac
  • 2Department of Pathology, Yonsei University College of Medicine, Seoul, Korea.
  • 3Department of Radiology, Yonsei University College of Medicine, Seoul, Korea.

Abstract

Eosinophilic fasciitis is a rare disease characterized by diffuse fasciitis with peripheral eosinophilia and progressive induration and thickening of the skin and soft tissues. We report a 19-year-old female who presented with pitting edema in both lower extremities. She had a history of excessive physical activity before her symptoms developed. Physical examination revealed 2+ pitting edema in both lower legs. She complained of mild pain in both knee joints and feet, with no tenderness or heating sensations. Laboratory results were unremarkable except for severe eosinophilia. Parasite infection, venous thrombosis, and cardiac and renal problems were excluded. A magnetic resonance imaging study of both lower extremities revealed increased signal intensity in the subcutaneous lesions, consistent with superficial inflammation of the fascia. Mixed perivenular lymphoplasmacytic and eosinophilic infiltration in the subcutaneous lesion were observed on biopsy. The patient was treated with corticosteroids, resulting in remarkable improvement in both edema and eosinophilia.

Keyword

Eosinophilia; eosinophilic fasciitis; pitting edema

MeSH Terms

Adrenal Cortex Hormones
Biopsy
Edema*
Eosinophilia
Eosinophils*
Fascia
Fasciitis*
Female
Foot
Hot Temperature
Humans
Inflammation
Knee Joint
Leg
Lower Extremity*
Magnetic Resonance Imaging
Motor Activity
Physical Examination
Rare Diseases
Skin
Young Adult
Adrenal Cortex Hormones

Figure

  • Fig. 1 Fat-saturated T2-weighted magnetic resonance imaging of the lower thigh of a patient with eosinophilic fasciitis. Arrows indicate inflammatory changes in the superficial fascia and subcutaneous fat layer.

  • Fig. 2 Perivascular eosinophilic infiltration in deep dermal and subcutaneous fat tissue (×400).


Reference

1. Bousquet J, Chanez P, Lacoste JY, Barnéon G, Ghavanian N, Enander I, Venge P, Ahlstedt S, Simony-Lafontaine J, Godard P. Eosinophilic inflammation in asthma. N Engl J Med. 1990; 323:1033–1039.
2. Oehling AG Jr, Walker C, Virchow JC, Blaser K. Correlation between blood eosinophils, T-helper cell activity markers and pulmonary function in patients with allergic and intrinsic asthma. J Investig Allergol Clin Immunol. 1992; 2:295–299.
3. Ciprandi G, Cirillo I, Vizzaccaro A, Milanese M, Tosca MA. Airway function and nasal inflammation in seasonal allergic rhinitis and asthma. Clin Exp Allergy. 2004; 34:891–896.
4. Heath H, Qin S, Rao P, Wu L, LaRosa G, Kassam N, Ponath PD, Mackay CR. Chemokine receptor usage by human eosinophils. The importance of CCR3 demonstrated using an antagonistic monoclonal antibody. J Clin Invest. 1997; 99:178–184.
5. Tillie-Leblond I, Hammad H, Desurmont S, Pugin J, Wallaert B, Tonnel AB, Gosset P. CC chemokines and interleukin-5 in bronchial lavage fluid from patients with status asthmaticus. Potential implication in eosinophil recruitment. Am J Respir Crit Care Med. 2000; 162:586–592.
6. Ying S, Robinson DS, Meng Q, Rottman J, Kennedy R, Ringler DJ, Mackay CR, Daugherty BL, Springer MS, Durham SR, Williams TJ, Kay AB. Enhanced expression of eotaxin and CCR3 mRNA and protein in atopic asthma. Association with airway hyperresponsiveness and predominant co-localization of eotaxin mRNA to bronchial epithelial and endothelial cells. Eur J Immunol. 1997; 27:3507–3516.
7. Ma W, Bryce PJ, Humbles AA, Laouini D, Yalcindag A, Alenius H, Friend DS, Oettgen HC, Gerard C, Geha RS. CCR3 is essential for skin eosinophilia and airway hyperresponsiveness in a murine model of allergic skin inflammation. J Clin Invest. 2002; 109:621–628.
8. Blanchet MR, McNagny KM. Stem cells, inflammation and allergy. Allergy Asthma Clin Immunol. 2009; 5:13.
9. Rådinger M, Bossios A, Sjöstrand M, Lu Y, Malmhäll C, Dahlborn AK, Lee JJ, Lötvall J. Local proliferation and mobilization of CCR3(+) CD34(+) eosinophil-lineage-committed cells in the lung. Immunology. 2011; 132:144–154.
10. Daugherty BL, Springer MS. The beta-chemokine receptor genes CCR1 (CMKBR1), CCR2 (CMKBR2), and CCR3 (CMKBR3) cluster within 285 kb on human chromosome 3p21. Genomics. 1997; 41:294–295.
11. Lee JH, Chang HS, Kim JH, Park SM, Lee YM, Uh ST, Rhim T, Chung IY, Kim YH, Park BL, Park CS, Shin HD. Genetic effect of CCR3 and IL5RA gene polymorphisms on eosinophilia in asthmatic patients. J Allergy Clin Immunol. 2007; 120:1110–1117.
12. Garcia-Zepeda EA, Rothenberg ME, Ownbey RT, Celestin J, Leder P, Luster AD. Human eotaxin is a specific chemoattractant for eosinophil cells and provides a new mechanism to explain tissue eosinophilia. Nat Med. 1996; 2:449–456.
13. Rothenberg ME, MacLean JA, Pearlman E, Luster AD, Leder P. Targeted disruption of the chemokine eotaxin partially reduces antigen-induced tissue eosinophilia. J Exp Med. 1997; 185:785–790.
14. Pope SM, Fulkerson PC, Blanchard C, Akei HS, Nikolaidis NM, Zimmermann N, Molkentin JD, Rothenberg ME. Identification of a cooperative mechanism involving interleukin-13 and eotaxin-2 in experimental allergic lung inflammation. J Biol Chem. 2005; 280:13952–13961.
15. Shin HD, Kim LH, Park BL, Jung JH, Kim JY, Chung IY, Kim JS, Lee JH, Chung SH, Kim YH, Park HS, Choi JH, Lee YM, Park SW, Choi BW, Hong SJ, Park CS. Association of Eotaxin gene family with asthma and serum total IgE. Hum Mol Genet. 2003; 12:1279–1285.
16. Carlson CS, Eberle MA, Kruglyak L, Nickerson DA. Mapping complex disease loci in whole-genome association studies. Nature. 2004; 429:446–452.
17. Standards for the diagnosis and care of patients with chronic obstructive pulmonary disease (COPD) and asthma. This official statement of the American Thoracic Society was adopted by the ATS Board of Directors, November 1986. Am Rev Respir Dis. 1987; 136:225–244.
18. Crapo RO, Casaburi R, Coates AL, Enright PL, Hankinson JL, Irvin CG, MacIntyre NR, McKay RT, Wanger JS, Anderson SD, Cockcroft DW, Fish JE, Sterk PJ. Guidelines for methacholine and exercise challenge testing-1999. This official statement of the American Thoracic Society was adopted by the ATS Board of Directors, July 1999. Am J Respir Crit Care Med. 2000; 161:309–329.
19. Holland SM, Gallin JI. Disorders of granulocytes and monocytes. In : Braunwald E, Fauci AS, Kasper DL, Hauser SL, Longo DL, Jameson JL, editors. Harrison's principles of internal medicine. 15th ed. Vol. 1. New York (NY): McGraw-Hill;2001. p. 372.
20. Stephens M, Smith NJ, Donnelly P. A new statistical method for haplotype reconstruction from population data. Am J Hum Genet. 2001; 68:978–989.
21. Hedrick PW. Gametic disequilibrium measures: proceed with caution. Genetics. 1987; 117:331–341.
22. Bugawan TL, Mirel DB, Valdes AM, Panelo A, Pozzilli P, Erlich HA. Association and interaction of the IL4R, IL4, and IL13 loci with type 1 diabetes among Filipinos. Am J Hum Genet. 2003; 72:1505–1514.
23. Lou XY, Chen GB, Yan L, Ma JZ, Zhu J, Elston RC, Li MD. A generalized combinatorial approach for detecting gene-by-gene and geneby-environment interactions with application to nicotine dependence. Am J Hum Genet. 2007; 80:1125–1137.
24. Ritchie MD, Hahn LW, Roodi N, Bailey LR, Dupont WD, Parl FF, Moore JH. Multifactor-dimensionality reduction reveals high-order interactions among estrogen-metabolism genes in sporadic breast cancer. Am J Hum Genet. 2001; 69:138–147.
25. Brodie ED. Why evolutionary genetics does not always add up. In : Wolf JB, Brodie ED, Wade MJ, editors. Epistasis and the evolutionary process. New York (NY): Oxford University Press;2000. p. 3–19.
26. Pope SM, Zimmermann N, Stringer KF, Karow ML, Rothenberg ME. The eotaxin chemokines and CCR3 are fundamental regulators of allergen-induced pulmonary eosinophilia. J Immunol. 2005; 175:5341–5350.
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