Eosinophilic fasciitis induced by a game of drumming probably via type 2 innate immunity

Abstract

We report a case of eosinophilic fasciitis triggered by strenuous physical activity, which did not relapse during the follow‐up period. We ascertained that interleukin‐33 (IL‐33) was released from the vascular endothelial cells after intense exercise, inducing type 2 innate lymphocytes (ILC2) and causing fasciitis. A healthy woman experienced itching on both limbs a few hours after a game of drumming. Her hand, knee joints, and legs gradually swelled up with groove signs along the superficial veins. White blood cell and eosinophil counts were significantly elevated. Magnetic resonance imaging revealed a high signal at the fascia on both lower limbs. Histopathological findings of the left lower limb tissue specimen showed edematous fascia with eosinophils. No relapse of eosinophilic fasciitis was observed after finishing treatment with prednisolone. Immunological staining for IL‐4, IL‐5, IL‐33, tumor necrosis factor‐α, and interferon‐γ was performed on the fascial tissue. Both IL‐4 and IL‐5 were stained on the lymphocytes at the muscle and fascia levels; however, CD3 and CD4 were unstained in these cells, suggesting that those cells were ILC2. Tumor necrosis factor‐α and interferon‐γ were unstained. Vascular endothelial cells in the fascia strongly expressed IL‐33. Eosinophilic fasciitis may be associated with type 2 immunity triggered by IL‐33 in the current case.

Unmasking the Veiled Intruder: A Complicated Case of Eosinophilic Fasciitis

“…Most cases are deemed idiopathic, but EF is associated with infections, medications, autoimmune diseases, and hematologic diseases. Infections with SARS-CoV-2 and vaccinations against it, as well as immunotherapy with checkpoint inhibitors and strenuous exercise, have all been linked to EF as adverse effects [ 5 - 8 ]. The pathophysiology of EF has not been clearly established but is thought to involve activation of interleukin-5 (IL-5) and transforming growth factor-beta (TGF-β) [ 9 ].…”

Eosinophilic fasciitis following COVID-19: A case series of 3 patients

“…Eosinophilic fasciitis (EF) is a rare autoimmune connective tissue disorder characterized by inflammation and fibrosis of the fascia. 1 Sudden or gradual onset of erythematous, tender, symmetric edema followed by plate-like induration in the distal limbs is the typical clinical presentation. 2 Affected tissues later become bound down and display perifollicular dimpling.…”

“…Although the etiology of EF remains elusive, some potential triggers have been identified, including strenuous exercise, 1 infectious pathogens such as Borrelia burgdorferi , 4 hematologic disorders, autoimmune diseases, radiotherapy, and exposure to medications such as statins, ramipril, or immune checkpoint inhibitors. 5 Additionally, there have been several case reports published linking the development of EF to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination.…”

Dupilumab for treatment of eosinophilic fasciitis

“…also improvement in resident's confidence for the two knowledge-based questions [first question: 2.00 for pre and 4.00 (4) for post (P < 0.01); second question 2.00 for pre and 3.50 (4-3) for post (P < 0.01)]. There was no association between residents' knowledge before and after the curriculum (P = 0.49 and P = 1.00, respectively).…”

“…1,2 The latest evidence has shown that Type 2 innate immunity may give rise to EF. 3,4 Overactivity of IL-4/IL-13 signalling promotes the occurrence and perpetuation of fibrotic skin diseases. 5 We report a patient with EF successfully treated with methylprednisolone combined with dupilumab, a humanized IgG4 monoclonal antibody blocking signalling of T helper (Th)2 cytokines such as IL-4 and IL-13.…”