Eosinophilic fasciitis (EF), also known as Shulman syndrome, is a rare scleroderma-like disorder that is characterized by an acute onset of induration, swelling, erythema, and tenderness of the skin and deep fascia, often affecting all four limbs. We report a case of eosinophilic fasciitis in a 51-year-old female patient, whose diagnosis of EF was made based on the findings from clinical evaluation and magnetic resonance imaging (MRI) but without skin biopsy. She was treated with a combination therapy of prednisolone and methotrexate, and her response to therapy was assessed via clinical assessment and MRI. MRI may be a useful non-invasive diagnostic tool for not only supporting but also confirming the clinical diagnosis of EF when a skin-to-muscle biopsy is not available or cannot be performed, as well as for monitoring disease activity and response to therapy. Further prospective studies should be conducted to evaluate the precise sensitivity and specificity of MRI in diagnosing EF and also to create more structured protocols to guide the diagnosis and management of EF.

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Idiopathic inflammatory myopathies are mainly defined by inflammatory infiltrates within the muscle (lymphocytes and macrophages). Eosinophil muscle infiltration has been described in idiopathic eosinophilic myositis (IEM) and rarely in EF. This study aimed to further delineate the nosological frame of idiopathic eosinophil muscle infiltration through the exhaustive analysis of IEM and EF patients.
This multicentre retrospective case series included IEM patients diagnosed between 2000 and 2022. IEM inclusion criteria were eosinophilic muscle infiltration with myositis pathological features, after the exclusion of differential diagnoses. An additional group of EF patients diagnosed between 2016 and 2022 was constituted. Inclusion criteria were an EF diagnosis and fascia thickening with inflammatory infiltrate.
A total of 20 IEM cases and 10 EF cases were included. The median (interquartile range) age at diagnosis was 65 (49-70) years; there were 18 males. Data analysis delineated four subgroups: focal EM (FEM, n = 3), diffuse EM (DEM, n = 6), eosinophilic myofasciitis (EMF, n = 11) and EF (n = 10). FEM represented a limited and benign form of myositis. DEM cases presented objective muscle impairment with eosinophilic muscle infiltration. EMF patients presented subjective muscle impairment (myalgia, 55%), fasciitis (on histology and/or imaging), eosinophilic muscle infiltration and frequent hypereosinophilia (55%). EF patients presented myalgia (50%), muscle lesions on histology with fascia-restricted inflammatory infiltrates with (60%) or without (40%) eosinophils.
The analysis of IEM and EF patient characteristics delineates four subgroups (FEM, DEM, EMF and EF) in terms of clinical, laboratory, imaging, pathological and outcome specificities, and proposes an adapted diagnostic and care management approach.

Eosinophilic fasciitis (EF, also known as Shulman syndrome) is an uncommon connective tissue disease characterized by inflammatory thickening of the fasciae as well as swelling and hardening of the skin. It mostly affects the lower extremities. Swollen and indurated skin, together with the groove sign, are typical clinical signs. So far, biopsy evidence of inflammation and thickening of the fascia has been the gold standard for diagnosis. Magnetic resonance imaging (MRI) is mentioned in the literature as an alternative method for confirming the diagnosis. We present a case of asymmetric EF in a 54-year-old German male. He came with painful induration of the right forearm, with a characteristic groove sign and limitation of motion of the right hand. The blood count revealed eosinophilia with 0.57 G/l or 9.6% (normal: 0.05-0.5 G/l and 0.5-5.5%), ANA and ENA were negative. The diagnosis was confirmed histologically and we were able to detect a thickened fascia in MRI and ultrasound imaging. The EF also appeared in the left lateral malleolus during the course of the illness. Treatment was carried out with prednisolone and methotrexate.
UNASSIGNED: Die eosinophile Fasziitis (EF, auch Shulman-Syndrom) ist eine seltene Erkrankung des Bindegewebes mit entzündlicher Verdickung der Faszien sowie Schwellung und Verhärtung der Haut. Betroffen sind v. a. die distalen Extremitäten. Typische klinische Befunde stellen eine lokalisierte Schwellung und Verhärtung der Haut sowie das Groove-Sign (deutsch: Rillenzeichen/negatives Venenzeichen/Matratzenphänomen) dar. Der Goldstandard für die Diagnosesicherung ist bisher der bioptische Nachweis entzündlich verdickter Faszien. In der Literatur wird alternativ die Diagnosesicherung durch MRT-Bildgebung diskutiert. Wir berichten über einen Fall von asymmetrischer EF bei einem 54 Jahre alten, männlichen Deutschen. Die Vorstellung erfolgte mit schmerzhafter Verhärtung im Bereich des rechten Unterarms sowie charakteristischem Groove-Sign und einer Bewegungseinschränkung der rechten Hand. Im Blutbild zeigte sich eine Eosinophilie mit 0,57 G/l bzw. 9,6 % (norm. 0,05–0,5 G/l und 0,5–5,5 %), ANAs und ENAs waren negativ. Die Diagnosesicherung erfolgte histologisch, zusätzlich konnten wir verdickte Faszien sowohl im MRT als auch in der Sonographie nachweisen. Im Verlauf manifestierte sich die EF auch am linken Malleolus lateralis. Die Therapie erfolgte mit Prednisolon und Methotrexat.

This case report highlights the potentially underrecognized subtype of unilateral eosinophilic fasciitis (EF) in a 28 year old man. With fewer than 300 reported encounters to date, EF is a rare disease that eludes clinicians by presenting as a scleroderma like syndrome. As EF remains a clinical diagnosis, biopsy results may be nonspecific, and the disease can easily be misdiagnosed (or missed entirely) if a full thickness biopsy is not reviewed by a dermatopathologist. The authors also emphasize the importance of internationally accepted diagnostic criteria, of which at least two different sets exist.

Eosinophilic fasciitis (EF) is an uncommon autoimmune connective tissue disorder characterized by edema, erythema, and subsequent induration of the extremities. It is commonly treated with corticosteroids but there is no treatment ladder for immunosuppressants or steroid-sparing agents. We report two EF cases treated effectively with mycophenolate mofetil (MMF) or mycophenolic acid (MPA) and present a literature review. We performed a MEDLINE search using the keywords \'eosinophilic fasciitis\', \'Shulman syndrome\', \'mycophenolic acid\', or \'mofetil mycophenolate\', and found 8 articles with 27 cases in which MMF or MPA was used. Twenty-nine cases were reviewed (2 reported herein and 27 from the literature search); all patients received a combination of systemic corticosteroids and MMF. MMF/MPA were given as a steroid-sparing agent in 27 (93.1%), in 1 (3.4%) as adjunctive therapy with other immunosuppressants, and in one, as monotherapy 1 (3.4%). Nineteen had a complete response, 6, a partial response, and 2 were unresponsive to diverse immunomodulators; in 2 cases, the outcome was not reported. MMF and MPA show promising therapeutic results and could be a treatment option to reduce corticosteroid related side effects.

BACKGROUND: Eosinophilic fasciitis (EF) is a connective tissue disease with an unknown long-term course.
OBJECTIVE: To evaluate presence and determinants of residual disease damage in patients with EF after long-term follow-up.
METHODS: Patients with biopsy-proven EF were included for this cross-sectional study. Outcome measures included the Physician\'s Global Assessment of Disease Activity, Physician\'s Global Assessment of Damage (PhysGA-D), skin pliability scores, passive range of motion, and health-related quality of Life (HRQoL) questionnaires.
RESULTS: In total, 35 patients (24 of whom were female [68.6%]) with a median age of 60 years participated. All patients had detectable residual damage. Impairment of HRQoL, assessed by the Dermatology Quality of Life Index and the 36-Item Short-Form Survey, correlated to the extent of residual damage. The PhysGA-D score at participation correlated to signs of severe disease at presentation, such as increased C-reactive protein level (Spearman\'s rho [rs ] = 0.486, P = .006), involvement of the neck (rs = 0.528, P = .001) and trunk (rs = 0.483, P = .003), prolonged time to disease remission (rs = 0.575, P = .003), and presence of concomitant morphea (rs = 0.349, P = .040). Lastly, maximum methotrexate dose correlated negatively to PhysGA-D score at study participation (rs = -0.393, P = .022).
CONCLUSIONS: Sample size.
CONCLUSIONS: All patients with EF had detectable residual damage. Impairment of HRQoL correlated to the extent of residual damage. Advanced age and signs of severe disease at presentation were associated with the severity of residual damage.

BACKGROUND: Fasciitis with eosinophilia (FE), or Shulman syndrome, is a rare disease of unknown origin for which the nosological profile has not been clearly defined. It is clinically characterised by oedema and induration of the limbs with hypereosinophilia. It may be associated with morphea, in which case it carries a poor prognosis, or other diseases, particularly autoimmune conditions. Herein, we report a case of fasciitis associated with eosinophilia, morphea and vitiligo.
METHODS: A 45-year-old male patient followed up for vitiligo for 20 years had been presenting swelling and induration of the skin on all 4 limbs for the previous 7 months associated with morphea on the trunk. Treatment consisting of systemic corticosteroids and methotrexate was initiated and displayed a certain degree of efficacy.
CONCLUSIONS: The association of morphea/fasciitis with eosinophilia is a classical finding; the presence of vitiligo raises the question of possible association between these different disorders.

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