Juvenile eosinophilic fasciitis: a single center case series.

BACKGROUND: Eosinophilic fasciitis (EF) is a rare disease characterized by skin induration and musculoskeletal abnormalities. Diagnostic criteria for EF are based on adult populations. There is a need to expand the literature on EF in children due to limited reported cases and potential differences compared to adults. METHODS: We conducted a retrospective review of medical records for six pediatric patients diagnosed with EF at our institution between November 2011 and April 2023. Inclusion criteria required patients to be under 18 years of age at the time of diagnosis and to have confirmed diagnosis through clinical history, imaging, and histology. RESULTS: Most of our cohort were female (83%) and non-Hispanic white (50%). Age at diagnosis ranged from 4 to 16 years. Duration of symptoms before diagnosis varied from 1 to 12 months. Follow-up periods ranged from 14 to 123 months. Concurrent medical conditions included localized scleroderma, acquired thrombophilia, and juvenile idiopathic arthritis. Patients presented with progressive painful swelling, severe joint limitations, and positive prayer sign. Initial regimens involved corticosteroids and methotrexate. Hydroxychloroquine, immunoglobulin, mycophenolate mofetil, rituximab, and tocilizumab were also used depending on the patient's disease severity and course. CONCLUSIONS: Juvenile EF may manifest as swelling and progressive induration without apparent skin abnormalities. Unlike adult populations, no underlying malignancies or associations with trauma were observed in our cohort. Our cases did not exhibit systemic involvement observed in previous studies on juvenile EF. While non-specific, the prayer sign may aid in early recognition of juvenile EF and help prevent long-term disability.

Intravascular fasciitis of the scalp as a complication of ICP monitor placement: a case report and review of the literature.

BACKGROUND/IMPORTANCE: There are only 56 documented cases of intravascular fasciitis, a rare variant of nodular fasciitis. Of these cases, only 2 involved the scalp. This lesion is amenable to surgical resection, making it important to differentiate it from soft tissue malignancies of the scalp. CLINICAL PRESENTATION: We report an unusual case of intravascular fasciitis involving the scalp at the site of an intracranial pressure (ICP) monitor of a 13-year-old male patient. The lesion was surgically excised with no recurrence upon 1-month follow-up. CONCLUSION: Intravascular fasciitis is a benign, reactive proliferation of soft tissue that may arise at sites of prior trauma. It appears as a soft, painless, mobile lesion, and immunohistochemical studies are required to differentiate it from malignant lesions. The standard of care is surgical resection of the lesion.

Systemic autoimmune rheumatic diseases as paraneoplastic phenomena: 3 illustrative case reports and narrative review of the literature.

OBJECTIVES: Systemic autoimmune rheumatic diseases can occur as paraneoplastic phenomena in the context of underlying malignancies. We present three illustrative clinical cases and a narrative literature review focusing on systemic sclerosis, dermatomyositis and palmar fasciitis and polyarthritis syndrome. METHODS: Medical data of three patients from the University Hospitals Leuven were retrospectively and anonymously obtained and reviewed. A narrative review was performed, searching the databases of PubMed, Embase and Cochrane Library. RESULTS: Systemic sclerosis, dermatomyositis and palmar fasciitis and polyarthritis syndrome are systemic autoimmune rheumatic diseases that can present as paraneoplastic phenomena. Systemic autoimmune rheumatic diseases are often associated with the presence of specific autoantibodies, some associated with a high likelihood of underlying malignancy. The presence of anti-ribonucleic acid polymerase III antibodies and anti-transcription intermediary factor 1 gamma antibodies indicates an increased risk of underlying cancer in systemic sclerosis and dermatomyositis, respectively. Individual patient prognosis can be improved through early detection of underlying malignancy, hence the importance of adequate cancer screening. CONCLUSION: Some systemic autoimmune rheumatic diseases can appear as paraneoplastic phenomena, whereby the presence of specific autoantibodies is known to be related to the likelihood of underlying malignancy. We highlight the importance of clinician's knowledge of these distinct features, as it facilitates early detection and treatment of underlying malignancy, thereby improving individual patient prognosis.

[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.

Concomitant spinal dural arteriovenous fistula and nodular fasciitis in an adolescent: case report.

BACKGROUND: Spinal dural arteriovenous fistula (SDAVF) usually occurs during the 4th to 6th decades of life, and adolescent SDAVF is rarely reported. SDAVF arising around a tumor is also rare, and reported tumors are mostly schwannoma and lipoma. CASE PRESENTATION: We reported a 16-year-old male presented with progressive weakness and numbness of lower limbs for 3 months. A SDAVF was found, which was fed by right radicular arteries from segmental artery at L2 level and drained retrogradely into perimedullary veins. A concomitant spinal extradural nodular fasciitis at right L1/L2 intervertebral foramen was also noted. The SDAVF was completely obliterated by endovascular treatment and the tumor was debulked. The patient recovered well after the procedures. CONCLUSIONS: Our case report suggests SDAVF can occur in adolescent. The concomitant presence with a nodular fasciitis indicates that although it usually arises in subcutaneous tissue but can rarely form on the dura of spine.

Palmar Fasciitis and Polyarthritis Syndrome Associated with Lung Adenocarcinoma.

Palmar fasciitis and polyarthritis syndrome (PFPAS) is a rare paraneoplastic rheumatic disease with characteristic features. We herein report a 77-year-old man with lung adenocarcinoma and contralateral pulmonary metastasis receiving chemotherapy who presented with progressive symmetrical flexion contractures associated with palmar fascial thickening and arthritis of both hands and shoulders. He was diagnosed with PFPAS as paraneoplastic manifestations. Salazosulfapyridine was not effective, but 15 mg/day of oral prednisolone improved his symptoms. Physicians should consider PFPAS and rule out malignancy in patients with arthritis in the extremities and flexion contractures associated with palmar fascial thickening.

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.

A case of eosinophilic fasciitis without skin manifestations: a case report in a patient with lupus and literature review.

Eosinophilic fasciitis (EF) is a rare connective tissue disease that causes inflammation and fibrosis of the fascia, inducing pain and motor dysfunction. Characteristic skin manifestations, such as edema, erythema, induration, peau d'orange appearance, and the groove sign, are of diagnostic significance and observed in the majority of patients with EF. We herein report a case of EF without these characteristic skin manifestations. A 66-year-old Japanese woman developed progressive limb pain and motor dysfunction. No skin changes were observed. We diagnosed the patient with EF based on the clinical course, magnetic resonance imaging, and en bloc biopsy containing fascia and muscle. Oral prednisolone therapy markedly attenuated limb pain and motor dysfunctions. Through a systemic search of the medical literature, we retrieved 4 juvenile cases and 8 adult cases of EF without characteristic skin manifestations during the clinical course. We herein present a systemic review on EF without skin manifestations and discuss differences between the two proposed sets of diagnostic criteria of EF.

Unilateral carpal tunnel syndrome: an unusual presentation of nodular fasciitis.

Nodular fasciitis is a benign, self-limiting proliferative disorder of fibroblast of uncertain aetiology, occurs frequently in the forearm. Nodular fasciitis in hand inducing carpal tunnel syndrome is exceptional. There are four cases of non-intraneural nodular fasciitis causing peripheral neuropathy that has been reported previously. We present the case of a 38-year-old man with features of unilateral carpal tunnel syndrome. Decompression of the median nerve performed subsequently along with excision of the lesion in a piecemeal fashion. Histopathological and immunohistochemical findings were consistent with nodular fasciitis. There were complete resolution of symptoms and no sign of recurrence at the end of 1 year after surgery.

Eosinophilic fasciitis associated with generalized morphea and IgA nephropathy.

Eosinophilic fasciitis (EF) is a rare connective tissue disease characterized by increased peripheral blood eosinophils and diffuse fasciitis, generalized morphea (GM) is a subtype of localized scleroderma, and IgA nephropathy is a chronic glomerulonephritis caused by abnormal deposition of IgA in the mesangial area of the glomeruli. We describe a 49-year-old male patient with hard skin, cutaneous hyperpigmentation, and proteinuria. The patient had suffered from a long disease course of hard skin, while urine protein was newly detected. Finally, the clinical presentation and physical examination, limb MRI, skin biopsy, and renal biopsy confirmed the diagnosis of eosinophilic fasciitis associated with generalized morphea and IgA nephropathy. This case is the first report of EF associated with GM and IgA nephropathy.

Proliferative fasciitis: A rare cause of disturbances in an adolescent hand.

We report a case of hand soft tissue tumor-proliferative fasciitis (PF)-in a 12-year-old patient that presented as a painful lump causing trigger finger. After meticulous diagnostic workup, a surgical excision led to immediate amelioration of symptoms. PF is a rare benign pseudosarcomatous lesion arising typically in the subcutaneous tissue and fascia in adults. It is very uncommon in the hand. To the best of our knowledge, this is the first report of a trigger finger being caused due to this pathology. In this report, the authors review PF lesions on hands, advice careful evaluation of magnetic resonance imaging features, and recommend surgical management.

A case of eosinophilic fasciitis and generalized morphea overlap.

A 60-year old man developed skin hardening and edema on his extremities. Although he had been treated with oral prednisolone at another hospital, skin stiffness relapsed during tapering of prednisolone. At the initial visit to our department, physical examination showed skin hardening of the extremities and also symmetric erythematous macules on the back. Histological examination revealed fasciitis on the forearm and morphea on the back. Eosinophilic fasciitis is occasionally associated with morphea. However, cases of concurrent eosinophilic fasciitis and generalized morphea are rare. In the present case, CD34 was differentially expressed in both lesions, suggesting eosinophilic fasciitis and morphea are separate diseases with different origin of mesenchymal cells.