Rare cardiac tumour-nodular fasciitis.

Left ventricular tumour is a rare condition in children. The causes include vegetations, thrombus, and fibroma. 2-year-old asymptomatic female presented with an innocent heart murmur at 6 months of age. Subsequent follow-ups at 18 months of age showed left ventricular mass. Surgical pathology revealed "nodular fasciitis." This type of tumour has never been described in the heart before.

The first case of SARS-CoV-2-induced eosinophilic fasciitis.

Eosinophilic fasciitis (EF), also known as Shulman syndrome, is a rare auto-immune fibrosing disorder of the fascia. Etiopathogeny of EF is still unclear. Nowadays, it is widely known that severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) may induce hyper-stimulation of the immune system. Several cases with fasciitis and rhabdomyolysis induced by coronavirus disease 2019 vaccines have been reported in the literature. Herein, we report the first case of EF possibly triggered by SARS-CoV-2 infection. A 45-year-old Tunisian woman, with no medical history, presented to our department with severe widespread muscle pain noticed one month after a SARS-CoV-2 infection. Physical examination showed an induration of the skin and subcutaneous tissue of the arms, forearms and legs with a restricted joint mobility. The level of eosinophils was 430 E/mm3 (6.1%) [1-4%]. Electromyography and creatine kinase levels were normal. Myositis-related antibodies were negative. Magnetic resonance imaging of the left arm showed high-intensity signal and thickness of the fascia without evidence of muscle or bone involvement. A muscular biopsy from the right deltoid showed thickening and inflammation of the fascia. The patient received intraveinous injections of 1000 mg of methylprednisolone for 3 days with an oral relay of 1 mg/kg per day of prednisone equivalent during 4 weeks. At one-month follow-up, a significant improvement of the skin induration and myalgia was observed, with a disappearance of the biological inflammatory syndrome. This brief report suggests a potential link between SARS-CoV-2 infection and new-onset of auto-immune fasciitis.

Nodular Fasciitis and Myxolipoma of the Larynx: A rare case report with brief literature review.

Nodular fasciitis (NF) is a peculiar, rapid-growing soft tissue lesion, typically appearing in subcutaneous tissue. Approximately 20% of NF occurs in the head and neck region, where they can involve any anatomic site. Laryngeal involvement, however, is quite rare. Lipoma is recognised as a slow growing, benign mesenchymal tumour; myxolipoma is a rare variant which has a prominent myxoid background. Laryngeal lipoma is infrequent, accounting for only 0.6% of all benign laryngeal lesions. We report a 61-year-old male patient with laryngeal nodular fasciitis coexisting with myxolipoma who presented to a tertiary care hospital in Ar Ramtha, Jordan, in 2020. Radiological and histological findings were indicative of laryngeal nodular fasciitis and myxolipoma was incidentally diagnosed. Following trans-oral debulking of the lesion the mass enlarged rapidly and the patient underwent a tracheostomy with complete mass excision and right partial laryngectomy through an open surgical approach. The patient had an uneventful recovery with no evidence of recurrence. The purpose of this report is to broaden the differential diagnosis of rapid-growing laryngeal masses that cause airway obstruction and to stress the significance of integrative interdisciplinary collaboration to establish an accurate diagnosis, thereby allowing proper management for benign pathologies and avoid futile aggressive treatment.

Graft versus host disease-related eosinophilic fasciitis: cohort description and literature review.

BACKGROUND: Chronic graft versus host disease (cGVHD) simulating eosinophilic fasciitis (EF) is an underdiagnosed and challenging complication due to the lack of knowledge about its pathogenesis, refractoriness to traditional immunosuppressive agents and their negative impact on the physical function and quality of life. The aim of this study is to describe the clinical-biological characteristics and response to treatment of a case series and to provide a comprehensive literature review on cGVHD related EF involvement. METHODS: Prospective observational study to describe the clinical and diagnostic evaluation characteristics of patients with EF-like follow-up as part of our multidisciplinary cGVHD consultations. In addition, the literature on joint and/or fascial musculoskeletal manifestations due to cGVHD was comprehensively reviewed. RESULTS: 118 patients were evaluated in multidisciplinary cGVHD consultations, 39 of whom (33%) developed fasciitis. Notably, 11 patients had isolated joint contractures without sclerotic skin. After a median of three lines of treatment, the vast majority of patients achieved some degree of response. 94 potentially eligible articles were identified by the search strategy, with 17 of them, the majority isolated case reports, making the final selection. The validated staging scales used for the assessment were the Joint and Fascial Score and the Photographic Range of Motion. CONCLUSION: Fascial/articular involvement needs to be recognized and evaluated early. To our knowledge, our cohort is the second largest series to have been reported. Literature addressing fascial/joints complications related to cGVHD is scarce. The search for new biomarkers, the use of advanced imaging techniques and multidisciplinary approach may help improve the prognosis of patients with cGVHD.

[A case of localized fasciitis with ulcerative colitis].

A 36-year-old man with ulcerative colitis presented with bloody stools at the beginning of October 2020. His condition had been stable without treatment since diagnosis 4 years prior. He was administered 4,000 mg of salazosulfapyridine orally and the bloody stools resolved. Fifteen days after treatment, he was admitted to our hospital with swelling and pain in his right lower leg. Laboratory results revealed an elevated erythrocyte sedimentation rate (43 mm/hr) and mildly elevated C-reactive protein levels (4.08 mg/dl). His D-dimer level was also elevated at 7.6 µg/ml. MRI using fat saturated T2-weighted imaging demonstrated marked hyperintensity in the fascia of the lower leg flexor and blood vessels of interstitial. In gadolinium-enhanced T1-weighted images, the deep veins were found to be dilated and the vein walls and their surrounding areas strongly contrasted, suggestive of localized fasciitis. No abnormalities were found on biopsy of his right gastrocnemius muscle on the 5th day after admission. Two days after the muscle biopsy, the patient began experiencing swelling and pain in his left lower leg. The high intensity lesions in his right leg were reduced on MRI performed the same day, but that of the fascia between the left gastrocnemius and soleus muscles was noted. We administered 60 mg (1.0 mg/kg/day) of prednisolone orally on day 9 and the pain and swelling in both legs promptly resolved. The prednisolone was tapered to 5 mg/day and as of the time of writing, resolution of pain and swelling has been maintained. Gastrocnemius myalgia syndrome, which causes pain and localized fasciitis, is often reported as a complication of Crohn's disease but is rare in conjunction with ulcerative colitis. It is important that clinicians are aware of this syndrome so it can be recognized early and successfully treated.

Eosinophilic Fasciitis with Hypereosinophilia as the Initial Clinical Manifestation of Peripheral T-Cell Lymphoma, Not Otherwise Specified.

A 58-year-old man presented with painful edema of the extremities, and a diagnosis of eosinophilic fasciitis (EF) was confirmed. He also met the criteria for hypereosinophilic syndrome (HES), but there were no findings suggestive of malignancies or hematologic neoplasms despite a close examination. He was started on steroid therapy but subsequently developed severe liver dysfunction, hemophagocytic lymphohistiocytosis, hepatosplenomegaly, and renal involvement. The diagnosis of peripheral T-cell lymphoma, not otherwise specified was finally established by a bone marrow reexamination and liver biopsy. In cases of eosinophilia, EF, and/or HES, it is important to suspect an intrinsic abnormality, including potential T-cell lymphoma.

Gulf war illness, post-HPV vaccination syndrome, and Macrophagic Myofasciitis. Similar disabling conditions possibly linked to vaccine-induced autoimmune dysautonomia.

More than one-fourth of all Persian gulf war coalition soldiers remain seriously ill. Several epidemiological studies suggest a link between multiple vaccinations at the time of the military operation and the illness development. Macrophagic Myofasciitis and post-HPV vaccination syndrome are two newer controversial vaccine-related disabling ailments. OBJECTIVES: 1) To systematically review all original articles investigating the association of vaccines with gulf war illness, 2) To discuss gulf war illness, Macrophagic Myofasciitis, and post-HPV vaccination syndrome clinical similarities, 3) To discuss emergent pathogenetic mechanisms proposed for post-HPV vaccination syndrome that may be also relevant to gulf war illness and Macrophagic Myofasciitis. RESULTS: All original epidemiological studies (n = 11) found a positive association between vaccination and gulf war illness development. Chronic fatigue, widespread pain and cognitive impairment characterize the three syndromes under discussion. Anti-adrenergic receptor antibodies, dysautonomia and small fiber neuropathy have been recently described in patients with post-HPV vaccination syndrome. CONCLUSION: post-HPV vaccination syndrome, Macrophagic Myofasciitis, and gulf war illness analogy suggests that some vaccines or multiple vaccinations in a very short period of time may induce, in susceptible individuals, chronic pain, fatigue and dyscognition. Vaccine-induced autoimmune dysautonomia is hypothesized as the common pathogenetic mechanism for this symptom cluster. Further research on the presence of small fiber neuropathy, adrenergic receptor antibodies, and abnormal autonomic function tests in the three syndromes under discussion may help to elucidate this hypothesis.

Surgical Treatment of Oral Cavity Nodular Fasciitis.

BACKGROUND: Nodular fascitiis is a myofibroblastic neoplasm of the soft tissue that rarely affects oral cavity. With a broad pattern of presentation, sometimes Nodular Fascitiis can have a rapid growth and appear highly cellular with local aggressiveness on biopsies, thus simulating a sarcoma. The aim of this paper is to present a case of troublesome diagnosis of nodular fascitiis mimicking a Malignant Fibrous Histiocytoma, with the purpose of alert clinicians and pathologists on the difficulties that can be met in the differential diagnosis between these 2 lesions. A 42-year-old male presented an exophytic lesion on the cheek. After the excisional biopsy, histological and immunohistochemical evaluations revealed a picture of doubtful significance. With a careful analysis, the diagnosis of nodular fasciitis was made and the patient was not further treated. At a 3-year follow-up, no recurrence was found. Differential diagnosis within myofibroblastic neoplasm can be a real challenge for both Clinicians and Pathologist. A coordinated team-work is mandatory to avoid clinical malpractice and unnecessarily aggressive treatment.

Myalgia and chronic fatigue syndrome following immunization: macrophagic myofasciitis and animal studies support linkage to aluminum adjuvant persistency and diffusion in the immune system.

Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) is a multifactorial and poorly undersood disabling disease. We present epidemiological, clinical and experimental evidence that ME/CFS constitutes a major type of adverse effect of vaccines, especially those containing poorly degradable particulate aluminum adjuvants. Evidence has emerged very slowly due to the multiplicity, lack of specificity, delayed onset, and frequent medical underestimation of ME/CFS symptoms. It was supported by an epidemiological study comparing vaccinated vs unvaccinated militaries that remained undeployed during Gulf War II. Affected patients suffer from cognitive dysfunction affecting attention, memory and inter-hemispheric connexions, well correlated to brain perfusion defects and associated with a stereotyped and distinctive pattern of cerebral glucose hypometabolism. Deltoid muscle biopsy performed to investigate myalgia typically yields macrophagic myofasciitis (MMF), a histological biomarker assessing longstanding persistency of aluminum agglomerates within innate immune cells at site of previous immunization. MMF is seemingly linked to altered mineral particle detoxification by the xeno/autophagy machinery. Comparing toxicology of different forms of aluminum and different types of exposure is misleading and inadequate and small animal experiments have turned old dogma upside down. Instead of being rapidly solubilized in the extracellular space, injected aluminum particles are quickly captured by immune cells and transported to distant organs and the brain where they elicit an inflammatory response and exert selective low dose long-term neurotoxicity. Clinical observations and experiments in sheep, a large animal like humans, confirmed both systemic diffusion and neurotoxic effects of aluminum adjuvants. Post-immunization ME/CFS represents the core manifestation of "autoimmune/inflammatory syndrome induced by adjuvants" (ASIA).

Eosinophilic fasciitis: From pathophysiology to treatment.

Eosinophilic fasciitis is a disease originally proposed as "diffuse fasciitis with eosinophilia" by Shulman in 1974. The patients with this disease often have history of strenuous exercise or labor a few days to 1-2 weeks before the onset. The chief symptoms are symmetrical, full-circumference swelling and plate-like hardness of the distal limbs. This is accompanied by redness and pain in the early stages, with many cases exhibiting systemic symptoms such as fever or generalized fatigue. The lesions have been observed extending to the proximal limbs, though never on the face or fingers. En bloc biopsies from the skin to the fascia show marked fascial thickening and inflammatory cell infiltration by the lymphocytes and plasma cells. Eosinophilic infiltration is useful for the diagnosis but is only seen in the early stages of the disease. Recently, "Diagnostic criteria, severity classification, and clinical guidelines for eosinophilic fasciitis" were published. This review article discusses about eosinophilic faciitis in detail, from its pathophysiology to the treatment.

Ischemic Fasciitis of the Left Buttock in a 40-Year-Old Woman with Beta-Propeller Protein-Associated Neurodegeneration (BPAN).

BACKGROUND Ischemic fasciitis is a rare condition that occurs in debilitated and immobilized individuals, usually overlying bony protuberances. Because the histology shows a pseudosarcomatous proliferation of atypical fibroblasts, and because the lesion can increase in size, ischemic fasciitis can mimic sarcoma. Beta-propeller protein-associated neurodegeneration (BPAN) arises in infancy and is due to mutations in the WDR45 gene on the X chromosome. BPAN results in progressive symptoms of dystonia, Parkinsonism, and dementia once the individual reaches adolescence or early adulthood, and is usually fatal before old age. A case of ischemic fasciitis of the buttock is presented in an adult woman with BPAN. CASE REPORT A 40-year-old woman with BPAN and symptoms of mental and physical deterioration, had become increasingly wheelchair-dependent and presented with a mass in her buttock that had been increasing in size for two months. Computed tomography (CT) imaging showed an ill-defined subcutaneous lesion between the dermis and the gluteal muscle, which was suspicious for malignancy. A needle biopsy of the mass was performed. The histology examination showed benign ischemic fasciitis. A follow-up CT scan performed 3.5 months after identification of the lesion showed that it had decreased in size. CONCLUSIONS Ischemic fasciitis is a rare condition that is associated with immobility. Because BPAN is a neurodegenerative disease that can cause immobility, a history of BPAN in patients of all ages may be associated with an increased risk of developing ischemic fasciitis. The correct diagnosis is essential, as ischemic fasciitis, although benign, can mimic malignancy.