Table 2.

Laboratory, imaging, histopathology results, and therapy

Patient no.	Reference	Presentation and physical exam findings	CK, U/L	Peak absolute eosinophil count, per μL	Imaging	Biopsy	Treatment
1	Current paper	Elbows, wrists, knees, ankles, with tightness, swelling, reduced wrist range of motion, and a positive groove sign in the left leg and right forearm	933	3,500	MRI left tibia: very mild edema seen diffusely within the fascia	Full‐thickness skin biopsy: striking expansion of the fascia by connective tissue matrix comprising collagen and mucin. Superficial inflammatory cell infiltrate permeating the fascia comprising lymphocytes and plasma cells. CD3 staining demonstrates presence of T lymphocytes, CD8+ T cells predominate	Prednisone 60 mg/day, methotrexate 20 mg/week
2	Current paper	Pitting edema of hands and feet, myalgias; subsequent skin tightness of the forearms	<20	2,400	None performed	Full‐thickness skin biopsy: fibrosing stromal changes; subcutaneous, fascia, and skeletal muscle infiltration by plasma cell–rich inflammatory infiltrate with eosinophils; CD3+ staining demonstrates presence of T lymphocytes; CD4+ T cells predominate	Prednisone 60 mg/day, methotrexate 15 mg/week
3	Current paper	Swelling of volar aspect of forearms; limited knee range of motion	104	700	MRI right forearm: edema within the fascial planes of the extensor palmar compartments, muscle signal relatively maintained; mild wrist extensor and flexor tenosynovitis	None performed	Prednisone 80 mg/day, mycophenolate 3 g/day
4	Current paper	Fatigue, weight loss, extremity swelling initially; leathery texture of arms and legs; positive groove sign; reduced range of motion in fingers, wrists, elbows, shoulders, knees, and ankles		2,300	MRI pelvis: fascial edema in pelvis and proximal thighs, extensive soft tissue edema and proximal abductor muscles bilaterally	None performed	Prednisone 80 mg/day, methotrexate 15 mg/week
5	Khoja et al., 2016	Myalgia, puffiness of the face; thickened and tethered waxy skin on all limbs and abdomen	28	5,240	MRI right upper limb: marked fascial edema associated with the musculature of the arm, right chest wall involving the latissimus dorsi, serratus anterior, and pectoralis	Full‐thickness skin biopsy: infiltration of dermis with a lymphoeosinophilic infiltrate with scattered eosinophils in the interstitium	Methylprednisolone 1 g/day (for treatment of coincident presumed CNS vasculitis)
6	Lidar et al., 2018	Not reported	Not reported	Not reported	PET/CT: increased uptake in soft tissues in legs	Muscle biopsy: eosinophilic fasciitis	Corticosteroid and methotrexate
7	Andrés‐Lencina et al., 2018	Brownish‐red plaque with significant induration and tethered waxy skin on the pubis that extended to the left anterior iliac crest region	Not reported	4,000	None performed	Full‐thickness skin biopsy: dermal fibrosis with dense hyalinized collagen, extending to the subcutis and fascia. Lymphocytic perivascular infiltration was also observed, with eosinophils that deepened to fascia	Prednisone 100 mg/day; methotrexate 20 mg/week (cyclosporine ineffective)
8	Le Tallec et al., 2019	Myalgia, diffuse skin thickening of arms and legs	WNL	4,140	MRI left thigh: abnormal linear high signal along the fasciae	Muscle biopsy: marked CD8‐positive inflammatory infiltrate of the fasciae coexisting with eosinophils with an anecdotic muscle involvement, thus ruling out other diagnoses such as myositis	Corticosteroid and methotrexate
9	Toussaint et al., 2019	Myalgia, extremity edema; woody induration, peau d'orange, positive groove sign bilateral forearms	227	4,092	MRI bilateral forearms: superficial and deep fascial thickening, thickened skin, and intra‐/subcutaneous edema	None performed	Prednisone 1 mg/kg/day, methotrexate
10	Rischin et al., 2018	Diffuse arthralgia and myalgia; forearms and hands with skin tightness and forearm induration, severe limitation of finger movement	WNL	600	MRI forearm: fasciitis surrounding all muscle compartments with no myositis	Left forearm biopsy: florid lymphocytic fasciitis composed of predominantly T cells and macrophages, with scattered plasma cells and perivascular cuffing by lymphocytes in the deep subcutaneous tissue and underlying fascia; CD3+ staining demonstrates presence of T lymphocytes	Prednisolone 50 mg/day, methotrexate 20 mg/week
11	Parker et al., 2018	Fatigue and myalgia; proximal muscle weakness; woody feel to skin of forearms, contracture of the left forearm flexor	75	WNL	MRI: symmetric fascial thickening and intense STIR signal centered around the muscle fascia of all thigh and calf muscle groups	Full‐thickness skin‐muscle biopsy: fascial and perifascicular inflammatory infiltrate with CD3+ cells; majority of myofibers showed HLA Class‐I immunolabelling	Prednisolone 30 mg/day, IVIg
12	Daoussis et al., 2017	Swelling of wrists, knees, ankles, profound crepitus in all involved areas	Not reported	Not reported	MRI knee and ankle: symmetric myofasciitis with associated tenosynovitis	None performed	Methylprednisolone 12 mg/day
13	Narvaez et al., 2018	Both patients (patients 13 and 14) are described as having proximal symmetric leg weakness and stiffening of the skin of the lower extremities	Not reported	Not reported	MRI lower extremities: focal changes of myositis and fasciitis of the right gastrocnemius and soleus; subcutaneous edematous changes	None performed	NSAIDs and colchicine; drug withdrawal
14	Narvaez et al., 2018		Not reported	Not reported	MRI lower extremities: extensive changes of myositis and less marked changes of fasciitis involving the adductor muscles	None performed	Drug withdrawal
15	Bronstein et al., 2011	Clinically silent	WNL	Not reported	PET/CT: FDG‐avid abdominal fasciitis	None performed	Not reported

Abbreviations: CK, creatine kinase; CNS, central nervous system; FDG, fluorodeoxyglucose; IVIg, intravenous immunoglobulin; MRI, magnetic resonance imaging; NSAIDs, nonsteroidal anti‐inflammatory drugs; PET/CT, positron emission tomography/computed tomography; STIR, short tau inversion recovery; WNL, within normal limits.