Eosinophilic Fasciitis Illustrated by 18 F-FDG PET/CT.

ABSTRACT: Eosinophilic fasciitis is a rare sclerodermiform disease characterized by upper and lower limb edema. We present the case of a 71-year-old woman currently hospitalized for painful lower limb edema. Laboratory tests reveal moderate eosinophilia (0.8 g/L) and an inflammatory syndrome. Abdominopelvic ultrasound reveals no abnormalities. In light of the unexplained inflammatory syndrome, a 18 F-FDG PET/CT scan currently shows intense hypermetabolism of the fasciae in the lower and upper limbs. Following an MRI, a biopsy is performed, and the anatomopathology currently confirms eosinophilic fasciitis.

The clinical and imaging features of eosinophilic cystitis in children: a case series study.

BACKGROUND: Eosinophilic cystitis (EC) is rare in children and remains poorly understood. Our aim was to analyse the clinical and imaging features of eosinophilic cystitis in children. METHODS: A retrospective review of histologically confirmed eosinophilic cystitis between January 2008 and December 2022 was performed, including patient age, sex, symptoms, laboratory examination, radiology, treatment and outcome. RESULTS: Twelve children (two girls, 10 boys; age range: 3-12 years, mean age: 7.2 years) were included in the study. Urinary irritation symptoms (10/12), haematuria (5/12) and hypogastralgia (3/12) were the most common symptoms. Five patients had a history of allergies, six patients had elevated serum IgE, nine patients had elevated peripheral eosinophils and six patients had positive microscopic haematuria. Radiology revealed diffuse homogeneous or inhomogeneous thickening in seven patients, localised thickening in three patients, and solitary tumour-like lesions in the other two patients. Preservation of the mucosal line and bladder wall layering were observed in eleven patients, and perivesical exudation and small vessel dilatation were observed in ten patients. All four patients with delayed scans showed obvious delayed enhancement. One patient showed low signal intensity on T2-W imaging. All patients received antihistamine, antibiotic and/or corticosteroid therapy and two tumour-like patients underwent transurethral resection. Nine patients achieved complete response and three patients achieved partial response. CONCLUSION: The clinical and imaging manifestations of EC in children have relative characteristics; when urologist and radiologist confronted with similar cases, EC should be considered. The final diagnosis depends on pathological biopsy.

A Novel Air Trapping Segment Score Identifies Opposing Effects of Obesity and Eosinophilia on Air Trapping in Asthma.

Rationale: Density thresholds in computed tomography (CT) lung scans quantify air trapping (AT) at the whole-lung level but are not informative for AT in specific bronchopulmonary segments. Objectives: To apply a segment-based measure of AT in asthma to investigate the clinical determinants of AT in asthma. Methods: In each of 19 bronchopulmonary segments in CT lung scans from 199 patients with asthma, AT was categorized as present if lung attenuation was less than -856 Hounsfield units at expiration in ⩾15% of the lung area. The resulting AT segment score (0-19) was related to patient outcomes. Measurements and Main Results: AT varied at the lung segment level and tended to persist at the patient and lung segment levels over 3 years. Patients with widespread AT (⩾10 segments) had more severe asthma (P < 0.05). The mean (±SD) AT segment score in patients with a body mass index ⩾30 kg/m2 was lower than in patients with a body mass index <30 kg/m2 (3.5 ± 4.6 vs. 5.5 ± 6.3; P = 0.008), and the frequency of AT in lower lobe segments in obese patients was less than in upper and middle lobe segments (35% vs. 46%; P = 0.001). The AT segment score in patients with sputum eosinophils ⩾2% was higher than in patients without sputum eosinophilia (7.0 ± 6.1 vs. 3.3 ± 4.9; P < 0.0001). Lung segments with AT more frequently had airway mucus plugging than lung segments without AT (48% vs. 18%; P ⩽ 0.0001). Conclusions: In patients with asthma, air trapping is more severe in those with airway eosinophilia and mucus plugging, whereas those who are obese have less severe trapping because their lower lobe segments are spared.

Eosinophilic cystitis: a case report of a pseudotumoral lesion.

Eosinophilic cystitis is a rare inflammatory disorder of the bladder, characterized by eosinophilic infiltration of the bladder wall, fibrosis of the mucosa, and muscle necrosis. Ultrasonography, when performed, shows a pseudotumoral mass that can be mistaken for malignancy. We report a case of a woman referred to our department in which an ultrasound showed a bladder mass and the biopsy demonstrated the presence of eosinophilic cystitis. The patient was treated with medical treatment and the outcome was favorable with the disappearance of symptoms and no recurrence at controls. We carry a literature review of cystitis eosinophilic, the ultrasound appearance, and therapeutic modalities.

Application of ultrasound in a case of eosinophilic fasciitis mimicking stiff-person syndrome.

Eosinophilic fasciitis (EF) is a rare disorder characterized by muscle stiffness mimicking other neuromuscular diseases. The diagnosis of EF is made on the basis of typical skin lesions. We report a case of a 36-year-old male patient with suspected stiff-person syndrome (SPS), who presented with progressive limb muscle stiffness and limited mobility of both wrists without obvious skin changes. Ultrasound revealed fascial thickening of bilateral upper and lower limb muscles and enlargement of hypoechoic tissues around the flexor digitorum tendons of the wrist. Skin and fascia biopsy confirmed the diagnosis of EF. Prednisolone therapy resulted in the improvement of muscle stiffness and tightness. Our findings suggest the need to consider connective tissue diseases such as EF in a patient with atypical features of SPS. Ultrasound is helpful for visualizing the causes of muscle stiffness and joint contractures in EF patients.

Multiple Cerebral Infarctions Accompanied by Subcortical and Subarachnoid Hemorrhaging in Bilateral Border Zone Areas in a Patient with Eosinophilic Granulomatosis with Polyangiitis.

Eosinophilic granulomatosis with polyangiitis (EGPA) is often associated with peripheral neuropathy, but reports of central nervous system involvement are quite rare. We herein report a patient with EGPA first identified as having hypereosinophilia who later developed asthma, eosinophilic otitis media, sinusitis, and hemorrhagic colitis. She subsequently developed hemiparesis. Head magnetic resonance imaging revealed multiple cerebral infarctions with subcortical and subarachnoid hemorrhaging colocalized at the bilateral border zone areas. She was diagnosed with EGPA-induced stroke and successfully treated with oral prednisolone. Inflammation in the small cerebral arteries in EGPA may induce bilateral border zone infarction with colocalizing subcortical and subarachnoid hemorrhaging.

Eosinophilic fasciitis (Shulman disease) with clinical, imaging and pathological correlation.

Eosinophilic fasciitis (EF) is a rare subacute fibrosing disorder of unknown aetiology, characterised by thickening of the muscular fascia and subcutaneous tissue, leading to swelling of limbs and trunk and sparing fingers and toes. Eosinophilic infiltration and degranulation may prompt tissue damage and consequent fibrosis due to the accumulation of collagen and extracellular matrix proteins. MRI is the best imaging modality for diagnosis, depicting fascial thickening and enhancement. MRI may also have a significant role in excluding alternative diagnosis and guiding the skin-muscle biopsy.We report a case of EF with clinical and pathological correlation, highlighting the diagnostic value of MRI for early diagnosis and further treatment.

Response of eosinophilic fasciitis associated with Waldenström macroglobulinemia to rituximab.

Eosinophilic fasciitis (EF) and generalized morphea (GM) are rare and difficult-to-treat sclerosing skin diseases which may occur in association with hematologic disorders. We present a 66-year-old man with EF and associated Waldenström macroglobulinemia who received combination therapy with rituximab (375mg/m2 every other week, gradually extended to every eight weeks), prednisolone (1.25-30mg/d), and methotrexate (7.5-15mg/w). Three months after rituximab initiation, his skin condition improved steadily accompanied by a significant improvement in joint mobility with only mild and transitory flares (observation period: 59 months under treatment with rituximab). To date, there are five case reports on rituximab treatment of EF/GM with an association to hypergammaglobulinemia in three of those cases. Therapy effected significant improvement in four patients. Our case adds to the hitherto limited evidence that rituximab may be a promising therapeutic strategy for EF/GM in association with hypergammaglobulinemia.

Case Report: Challenges for the Diagnosis and Treatment of Strongyloides stercoralis in Chronic Obstructive Pulmonary Disease Patients.

Strongyloidiasis, a neglected tropical disease (NTD), which is caused by Strongyloides stercoralis, can be fatal in immunocompromised patients. In most chronic cases, infections most frequently are asymptomatic, and eosinophilia might be the only clinical characteristic of this disease. The use of corticosteroids in some diseases like chronic obstructive pulmonary disease (COPD) may lead to the development of the life-threatening S. stercoralis hyperinfection syndrome. In the present research, we presented five cases of strongyloidiasis with a history of COPD and receiving corticosteroids from Abadan County, southwestern Iran. By performing the direct smear stool examinations, two cases were identified and the other three cases were diagnosed using the agar plate culture method. Despite reporting eosinophilia in previous patients' hospitalizations, the fecal examination was not performed for parasitic infections. Moreover, pulmonary symptoms were similar, but gastrointestinal symptoms were varied, including nausea, vomiting, abdominal pain, epigastric pain, constipation, and diarrhea. All the included patients were treated with albendazole, which is the second-line drug for S. stercoralis, and relapse of infection was observed in two patients by passing few months from the treatment. The increased blood eosinophil count was shown to play important roles in both the management of COPD and diagnosis of helminthic infections. In COPD patients who are receiving steroids, screening and follow-up for strongyloidiasis should be considered as priorities. In addition, ivermectin, which is the first-line drug for strongyloidiasis, should be available in the region.

A rare case of eosinophilic cystitis involving the inside and outside of the urinary bladder associated with an infected urachal cyst.

BACKGROUND: Eosinophilic cystitis is a rare inflammatory disease of the bladder characterized by eosinophilic infiltration of the bladder wall. Most Eosinophilic cystitis cases present with mucosal lesions of the urinary bladder. We present a very rare case of large mass-forming eosinophilic cystitis, involving the inside and outside of the bladder associated with an infected urachal cyst. CASE PRESENTATION: A 59-year-old man presented with gross hematuria, fever, dysuria, and suprapubic pain. Computed tomography showed a heterogeneously enhancing mass that measured 7.6 cm × 4 cm located on the anterosuperior portion of the bladder with an internal fluid collection. Cystoscopy revealed a raspberry-like mass lesion on the bladder dome. Transurethral resection of the bladder was initially performed. The mass lesion protruding from inside the bladder was removed, and pus-like fluid was drained. The pathologic diagnosis was eosinophilic cystitis. Follow-up computed tomography showed a remnant mass outside the bladder and urachal cyst. To eliminate the remnant lesion, robot-assisted partial cystectomy was performed. The patient showed no evidence of recurrent disease on follow-up cystoscopy and computed tomography for up to 2 years. CONCLUSIONS: Clinicians should consider the possibility of eosinophilic cystitis in patients who present with hematuria, fever, and suprapubic pain and have both intravesical and extravesical masses.

Reverse butterfly pattern image with eosinophilia: Effective treatment with benralizumab-A case report.

A 16-year-old adolescent presented with dry cough, fever, weight loss, night sweats, exercise intolerance, and eosinophilia. Computed tomography showed consolidations with "reverse butterfly" pattern. He responded well to corticosteroids but had frequent relapses. He became steroid dependent and developed steroid related morbidity. Benralizumab was prescribed with complete resolution of eosinophilia and lung infiltrates with no adverse effect.