A Case of Eosinophilic Fasciitis Presenting with Pansclerotic Morphea

Eosinophilic fasciitis can be distinguished from scleroderma largely by a lack of Raynaud’s phenomenon, normal nailfold capillaroscopy & the absence of visceral organ involvement.

Discussion

Currently in the literature, several case reports describe patients with atypical presentations of eosinophilic fasciitis involving the face and hands, but we believe our case is the first describing a patient presenting with diffuse, pansclerotic, scleroderma-like skin involvement.⁵

Acknowledging that a wide diversity of location and degree of skin involvement beyond the typically indurated extremities exists is especially relevant considering the suggestion that joint contractures are the main morbidity associated with eosinophilic fasciitis. Contractures occur in 76% of untreated patients.^2,6

Our patient was not referred to a rheuma­tologist until about one year into her illness and had a diagnostic delay of 23 months. A delay of just six months has been significantly associated with a 14.7 times greater risk of poor treatment response.² Also, both trunk involvement and concurrent morphea are associated with more severe manifestations of disease. Patients suffering from morphea have a 1.9-fold greater risk of corticosteroid resistance and are three times as likely to require additional immuno­suppressive medications.²

Even further striking is the suggestion that the presence of morphea alone may represent a subset of eosinophilic fasciitis patients who are more likely to suffer from residual damage regardless of a diagnostic delay.⁶ This underlines the importance of having a high index of suspicion for eosinophilic fasciitis in patients presenting with morphea and obtaining full-thickness biopsies when eosinophilic fasciitis is on the differential diagnosis.

Conclusion

Eosinophilic fasciitis is a relatively rare diagnosis with a presentation that is increasingly recognized to be diverse, but as of yet has no codified diagnostic criteria.³ This combination makes its diagnosis particularly challenging, especially in the face of a presentation—such as in our patient—of an unusual degree of skin involvement, which makes it easy to mistake eosinophilic fasciitis for an alternative scleroderma mimic, like scleromyxedema.²  

Julia Munchel, MD, is a third-year postgraduate, studying internal medicine at Dartmouth-Hitchcock Medical Center, Lebanon, N.H.

William Monaco, MD, works as a rheumatologist at Maine General Medical Center in Augusta.

References

1. Lakhanpal S, Ginsburg WW, Michet CJ, et al. Eosinophilic fasciitis: Clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum. 1988 May;17(4):221–231.
2. Mazori DR, Femia AN, Vleugels RA. Eosinophilic fasciitis: An updated review on diagnosis and treatment. Curr Rheumatol Rep. 2017 Nov 4;19(12):74.
3. Bischoff L, Derk CT. Eosinophilic fasciitis: Demographics, disease pattern and response to treatment: Report of 12 cases and review of the literature. Int J Dermatol. 2008 Jan;
   47(1):29–35.
4. Morgan ND, Hummers LK. Scleroderma mimickers. Curr Treatm Opt Rheumatol. 2016 Mar;2(1):69–84.
5. Pardos-Gea J. Positive prayer sign in eosinophilic fasciitis. Rheumatology (Oxford). 2017 Apr 1;56(4):628.
6. Suzuki S, Noda K, Ohira Y, et al. Finger stiffness or edema as presenting symptoms of eosinophilic fasciitis. Rheumatol Int. 2015 Oct;35(10):1769–1772.