Eosinophilic Fasciitis: Current and Remaining Challenges
Abstract
:1. Introduction
2. Etiology
- strenuous exercise [1];
- some of the autoimmune diseases (systemic lupus erythematosus, Sjögren syndrome, primary biliary cirrhosis, thyroid disease);
- hematologic disorders (such as immune-mediated anemia or thrombocytopenia, pancytopenia, aplastic anemia, pure red cell aplasia, Hodgkin lymphoma, myelomonocytic leukemia, chronic lymphocytic leukemia, other leukemias and lymphomas, multiple myeloma, and other myeloproliferative disorders); cancers [melanoma, lung]
- infection with Borrelia burgdorferi [10];
- use of adulterated rapeseed oil (epidemic outbreak in Spain in 1980s)
- radiotherapy
- graft-versus-host disease [11]
3. The Pathogenesis of EF
4. Histology
5. Clinic
6. Investigations
7. Diagnostic Criteria and Disease Severity
8. Differential Diagnosis
9. Treatment and New Potential Targets for EF
10. Discussion
11. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Major Criteria | Minor Criteria |
---|---|
Symmetrical plate-like sclerotic lesions present on the four limbs. | 1. The histology of a skin biopsy that incorporated the fascia shows fibrosis of the tissue, with thickening of the fascia and cellular infiltration of eosinophils and monocytes. |
2. Thickening of the fascia is seen using imaging tests, such as magnetic resonance imaging (MRI) |
Joint Involvement | Points |
---|---|
Joint contracture (upper limbs) | 1 point |
Joint contracture (lower limbs) | 1 point |
Limited movement (upper limbs) | 1 point |
Limited movement (lower limbs) | 1 point |
Expansion and worsening of skin rash (progression of symptoms) | 1 point |
A total of 2 or more points is classified as severe. |
Skin Changes (Erythema, Swelling, Induration) + Peripheral Eosinophilia |
---|
Exclude other disorders: |
Mono skin lesion:
|
Diffuse skin lesions:
|
No systemic involvement ↓ |
Proceed to full thickness skin-to-muscle biopsy or MRI of the affected area |
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Mazilu, D.; Boltașiu, L.A.; Mardale, D.-A.; Bijă, M.S.; Ismail, S.; Zanfir, V.; Negoi, F.; Balanescu, A.R. Eosinophilic Fasciitis: Current and Remaining Challenges. Int. J. Mol. Sci. 2023, 24, 1982. https://doi.org/10.3390/ijms24031982
Mazilu D, Boltașiu LA, Mardale D-A, Bijă MS, Ismail S, Zanfir V, Negoi F, Balanescu AR. Eosinophilic Fasciitis: Current and Remaining Challenges. International Journal of Molecular Sciences. 2023; 24(3):1982. https://doi.org/10.3390/ijms24031982
Chicago/Turabian StyleMazilu, Diana, Laura Alina Boltașiu (Tătaru), Denise-Ani Mardale, Maria Silviana Bijă, Sermina Ismail, Violeta Zanfir, Florentina Negoi, and Andra Rodica Balanescu. 2023. "Eosinophilic Fasciitis: Current and Remaining Challenges" International Journal of Molecular Sciences 24, no. 3: 1982. https://doi.org/10.3390/ijms24031982