Eosinophilic fasciitis in eosinophilic granulomatosis with polyangiitis

EF and eosinophilic granulomatosis with polyangiitis (EGPA) are two rare inflammatory disorders characterized by eosinophilic infiltration of the fascia of the distal limbs and multi-systemic eosinophilic granulomatosis with small-to-medium vessel vasculitis, respectively [1, 2]. Here we report two cases of ANCA-negative EGPA complicated by EF. A 35-year-old patient developed painful indurations of the lower legs 10 years after EGPA onset. MRI revealed panniculitis and fasciitis (Fig. 1). Biopsy disclosed septal panniculitis and EF. In a 22-year-old patient, histologically confirmed EF preceded the diagnosis of EGPA by 9 years. Both patients shared frequent EGPA manifestations, i.e. asthma, pulmonary infiltrates, and hypereosinophilia. Furthermore, the first patient was affected by perimyocarditis. After non-lasting responses to cytotoxic immunosuppressants, remission was induced with the monoclonal anti-IL-5 antibody mepolizumab in both patients. While panniculitis has been reported in EGPA, there have been no reports on EF as a manifestation of EGPA to date [1, 2]. Given the temporal link between EF and EGPA onset shown here—albeit long separated in time—and the role of IL-5 for eosinophil activation and differentiation in both diseases, EF appears to represent a preceding or complicating manifestation in rare cases of EGPA.

Figure 1.

(A) Induration, discolouration and patient-inflicted excoriation on lower legs. (B) Axial MRI showing oedema and contrast medium uptake in subcutis in T2-STIR sequence on the left and T1 sequence on the right proximal lower legs. (C) Axial MRI showing oedema and contrast medium uptake in subcutis and fascia in T2-STIR-sequence on the left and T1-sequence on the right distal lower legs

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The data underlying this article are available in the article.

Funding

No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this article.

Disclosure statement: O.H., S.A. and I.D. declare that there are no conflicts of interests. P.L. received consulting fees and other payments from AstraZeneca, Novartis, Vifor Pharma and GSK. P.L. participated on Advisory Boards of AstraZeneca, Novartis, Vifor Pharma and GSK. P.L. received grants from German Research Society, Federal Ministry of Education and Research, German Society of Rheumatology and Vifor Pharma for his institution.

Ethics: Informed consent has been obtained for publication of this vignette.

References

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