Immune checkpoint inhibitor-induced eosinophilic fasciitis: a case report and literature review / 中华内科杂志

A 58-year-old male patient with angioimmunoblastic T-cell lymphoma developed a rash and skin tightness on the face, limbs, and trunk together with joint stiffness and dysfunction after 6 months of treatment with the programmed cell death protein-1 inhibitor camrelizumab. Laboratory tests revealed progressive eosinophilia over 6 months, with the eosinophil count increasing from 0.07×10 9/L to 3.3×10 9/L. Magnetic resonance imaging showed thickened skin of both forearms, while T 2-weighted imaging showed markedly increased signal intensity within the myofascia. Skin biopsy of the right forearm showed thickened and fibrosed fascia and infiltration of inflammatory cells, including lymphocytes, plasma cells, and eosinophils. The patient was diagnosed with immune checkpoint inhibitor (ICI)-induced eosinophilic fasciitis (EF). After beginning treatment with methylprednisolone (40 mg daily), methotrexate (10 mg/week), and baricitinib (4 mg daily), his symptoms of skin tightness and joint dysfunction significantly improved within 1 month, and his peripheral blood eosinophil count decreased to 0.17×10 9/L. ICI-induced EF is a rare immune-related adverse reaction. To date, only 20 cases have been reported in published foreign literature, and their clinical characteristics are summarized here. The time from ICI treatment to EF was 12 (8,15) months, and the main clinical manifestations included skin involvement ( n=19), joint dysfunction ( n=11), myalgia/muscle weakness ( n=9), and peripheral eosinophilia ( n=16). After treatment, the clinical symptoms of EF improved in 17 patients, and eosinophil counts returned to normal after 3 (1,8) months. EF is a dysfunctional adverse response to ICI therapy. Tumor patients undergoing immunotherapy should be monitored for symptoms of EF. Early treatment is essential for preventing complications.

Clinical and imaging characteristics of 107 patients with linear morphea: a retrospective analysis / 中华皮肤科杂志

Objective:To analyze clinical, laboratory and imaging characteristics of different subtypes of linear morphea (LM) , and to propose an appropriate approach to the diagnosis and severity assessment of LM.Methods:Clinical data were collected from patients with clinically and/or pathologically confirmed LM in Department of Dermatology, West China Hospital, Sichuan University from January 2018 to December 2019, and retrospectively analyzed.Results:A total of 107 patients with LM were enrolled into this study, including 63 with LM of the limbs/trunk, 22 with morphea en coup de sabre, 11 with progressive hemifacial atrophy and 11 with eosinophilic fasciitis. Disease severity was evaluated by using the modified localized scleroderma skin severity index (mLoSSI) and localized scleroderma skin damage index (LoSDI) scores in 88 patients, with the mLoSSI scores ranging from 0 to 51 points, and the LoSDI scores ranging from 0 to 40 points. Routine blood examination of 10 patients with eosinophilic fasciitis showed increased eosinophil counts in 4 patients. Thirteen (14.8%) of 88 patients with LM were positive for antinuclear antibody, with titers of ≥ 1∶320. Magnetic resonance imaging (MRI) examination showed ipsilateral cerebral hemisphere atrophy and contralateral white matter hyperintensity on T2-weighted images in 2 out of 4 patients with progressive hemifacial atrophy, myofascial thickening in 26 out of 28 patients with LM of the limbs/trunk (92.9%) , subcutaneous septal and myofascial thickening in all 11 patients with eosinophilic fasciitis.Conclusions:The preliminary assessment of disease activity, severity and prognosis of LM can be made by mLoSSI and LoSDI. MRI examination is recommended for patients with clinical signs of involvement of subcutaneous structures.

Fascitis eosinofílica: reporte de un caso / Eosinophilic fasciitis: a case report

RESUMEN La fascitis eosinofílica es una enfermedad rara del tejido conectivo que se caracteriza por induración y engrosamiento progresivo y simétrico de la piel y del tejido celular subcutáneo localizado, principalmente, en las extremidades. Además de las manifestaciones cutáneas hay compromiso articular, muscular y, en casos excepcionales, compromiso sistémico. Su diagnóstico se basa en los hallazgos clínicos, eosinofilia en sangre periférica y la toma de una biopsia profunda de piel, que incluya la fascia donde se evidencia un infiltrado compuesto por linfocitos y eosinófilos. El tratamiento de elección son los esteroides sistémicos acompanados de medicamentos inmunosupresores.

ABSTRACT Eosinophilic fasciitis is a rare connective tissue disease. It is characterised by a progressive and symmetrical induration and thickening of the skin and soft tissues of the limbs. In addition to the skin manifestations, the joints and muscles are also involved, and in rare cases there can be systemic involvement. The diagnosis of EF is based on clinical findings, the presence of peripheral blood eosinophilia, and a full-thickness biopsy that should include the deep fascia in order to show the inflammatory infiltration that is mostly composed of lymphocytes and eosinophils. Systemic corticosteroids remain the treatment of choice and may be combined with an immunosuppressive drug.

Atypical presentations of eosinophilic fasciitis.

Eosinophilic fasciitis is an uncommon connective tissue disease that may mimic and overlap with other sclerosing disorders such as morphea and lichen sclerosus. Herein, we report four patients (two men and two women, aged 16-64 yeas) with eosinophilic fasciitis. There was overlap with both morphea and lichen sclerosus in 2 patients and with morphoea alone in 1 patient. Magnetic resonance imaging (MRI) was used for diagnosis in three patients and for assessing treatment response in one patient. Eosinophilic fasciitis may co-exist with morhoea and lichen sclerosus. In view of the overlapping clinical and histopathological features of these disorders, MRI may be helful in delineating the conditions by detecting involvement of fascia.

Eosinophilic Fasciitis And Associated Late Emerging Hodgkin Lymphoma.

Abstracts: Eosinophilic fasciitis is often seen between the ages of 40-50, and is characterized with skin thickening, and etiopathogenesis is still unclear. Patients diagnosed as eosinophilic fasciitis have accompanying hematologic diseases in less than 10 %. In cases reported previously in the literature with a diagnosis of eosinophilic fascitis, accompanying hematological malignancies and solid organ malignancies were within the first 5 years after diagnosis. We here present, in this case 58 years old male who diagnosed hodgkin lymphoma after 21 years of eosinophilic fasciitis.

Fascitis eosinofílica: a propósito de un caso / Eosinophilic fasciitis: an account of a case

Eosinophilic fasciitis is a rare entity characterized by induration of skin and peripheral eosinophilia. The pathogenesis is still not well known, but is known association with hematological, autoimmune or neoplastic diseases. The complex and not well standardized treatment. For a patient of 66 years presented with a history of morphea...

La Fascitis Eosinofílica (FE) es una entidad muy infrecuente, caracterizada por induración de piel y eosinofilia periférica. La patogenia aún no es bien conocida, pero es sabida la asociación con enfermedades hematológicas, autoinmunes o neoplásicas. El tratamiento es complejo y no bien estandarizado. Se presenta el caso de una paciente de 66 años, con antecedentes de morfea...

Fascitis eosinofílica: a propósito de un caso / Eosinophilic fasciitis: a case report

Presentamos el caso de una mujer de 71 años de edad, que consulta en el Servicio de Dermatología del Hospital por placas eritematosas, edematosas, induradas al tacto en antebrazos y piernas, que progresan con franca induración del tegumento, limitación de la movilidad articular, contracturas en flexión y debilidad muscular leve, asociado a placas esclerodermiformes en tronco. La histopatología informó epidermis y dermis conservada con fascitis eosinofílica evolucionada asociada a miositis. Se revisaron las formas de presentación clínica, los métodos diagnósticos (histopatología y resonancia magnética), diagnósticos diferenciales y posibles tratamientos.

We report the case of a 71 years old woman, consulting in the Dermatology Department of our hospital for erythematous, edematous, indurated to the touch patches in forearms and legs, progressing to frank induration of the integument, limited joint mobility, flexion contractures and mild muscle weakness associated with scleroderma-like plaques on the trunk. The histopathology reported epidermis and dermis preserved with eosinophilic fasciitis and myositis. The clinical presentation, diagnostic methods (histopathology and MRI), differential diagnosis and possible treatments, were reviewed.

A Case of Eosinophilic Fasciitis Presenting as Pitting Edema of the Lower Extremities

Eosinophilic fasciitis is a rare disease characterized by diffuse fasciitis with peripheral eosinophilia and progressive induration and thickening of the skin and soft tissues. We report a 19-year-old female who presented with pitting edema in both lower extremities. She had a history of excessive physical activity before her symptoms developed. Physical examination revealed 2+ pitting edema in both lower legs. She complained of mild pain in both knee joints and feet, with no tenderness or heating sensations. Laboratory results were unremarkable except for severe eosinophilia. Parasite infection, venous thrombosis, and cardiac and renal problems were excluded. A magnetic resonance imaging study of both lower extremities revealed increased signal intensity in the subcutaneous lesions, consistent with superficial inflammation of the fascia. Mixed perivenular lymphoplasmacytic and eosinophilic infiltration in the subcutaneous lesion were observed on biopsy. The patient was treated with corticosteroids, resulting in remarkable improvement in both edema and eosinophilia.

Report of 16 cases of eosinophilic fasciitis with literature review / 中华风湿病学杂志

Objective We reported 16 eosinophilic fasciitis (EF) patients with eosinophilic fasciitis and performed a systematic review of the literature to improve the disease awareness.Methods The clinical course of 16 patients with eosinophilic fasciitis at the Peking Union Medical College Hospital were described,inclu-ding demographic data,clinical manifest-ations,laboratory tests,pathology and treatment.Results The mean age at diagnosis was (47±8) years,with 13 female and 3 male patients.Three cases had exertion or strenuous sports before the onset of EF.Positive ANA was noted in 6 of 12,positive RF was noted in 3 of 10,hyper-gammaglobulinemia was noted in 6 of 7,elevated IgG was noted in 8 of 13,peripheral blood eosinophilia was noted in 10 of 16,while thrombocytopenia was found in one patient.Conclusion Based on this and other reported cases in the literature,EF may be a kind of autoimmune disease.Genetic influence and environ-mental factors are involved in the development of this disease.Systemic involvement is rare.In general,corticosteroids and immunosuppressive are effective in EF.

Eosinophilic Fasciitis: What Matters in Management in a Developing Country—A Case Report with Two and a Half-year Follow-up.

Eosinophilic fasciitis is an uncommon disorder of unknown aetiology and poorly-understood pathogenesis. Since 1974, over 250 cases of eosinophilic fasciitis have been reported worldwide. The first case of eosinophilic fasciitis from Bangladesh is reported here. The challenges of diagnosis, treatment, and follow-up, including family and social support, are discussed.

Eosinophilic Fasciitis as the Initial Manifestation of Graft-Versus-Host Disease

Eosinophilic fasciitis (EF) is a relatively rare disorder characterized by eosinophilia of the peripheral blood and inflammation of fascia and skin. The etiology of EF is unknown but it can precede or occur concomitantly with hematologic disorders including malignancy. EF can rarely occur after bone marrow transplantation and is a feature of chronic graft-versus-host disease (cGVHD). We report a 49-year-old man who developed EF as the first manifestation of cGVHD after allogeneic hematopoietic stem cell transplantation (AlloHSCT).

A Case of Steroid and Methotrexate-Resistant Eosinophilic Fasciitis Treated with Adalimumab

Eosinophilic fasciitis (EF) is a rare fibrosing disorder characterized by painful swelling and induration of the limbs and trunk, characteristic histology with sclerosis and lymphocytic inflammation affecting the fascia. The cause and pathogenesis of EF are still unknown and current therapies include glucocorticoids with or without use of immunosuppressive agents. Recently, there have been several case reports documenting the efficacy of a TNF alpha inEosinophilic fasciitis (EF) is a rare fibrosing disorder characterized by painful swelling and induration of the limbs and trunk, characteristic histology with sclerosis and lymphocytic inflammation affecting the fascia. The cause and pathogenesis of EF are still unknown and current therapies include glucocorticoids with or without use of immunosuppressive agents. Recently, there have been several case reports documenting the efficacy of a TNF alpha inhibitor in EF following a steroid-resistant disease course. However, there has been no report on the experience in treatment of EF with a TNF alpha inhibitor in Korea. Hence, we report a case of steroid and methotrexate-resistant EF which was successfully treated with adalimumab, along with a review of the relevant articles.

Two Cases of Eosinophilic Fasciitis

Eosinophic fasciitis (EF) is an uncommon connective tissue disease characterized by scleroderma-like cutaneous changes, peripheral eosinophilia, hypergammaglobulinemia, and an elevated erythrocyte sedimentation rate (ESR). Typical histopathologic findings include chronic inflammatory infiltration affecting the deep fascia with lymphocytes, histiocytes, and occasionally eosinophils. We report two cases of EF, the first of which is a 36-year-old man with a tender brownish induration on both forearms, for 2 months. Histopathologic examination showed fibrotic fascia with a mixed inflammatory cell infiltration. The second case is a 52-year-old woman with a symmetrical painful swelling and skin induration on both forearms, for 4 months. A deep biopsy demonstrated chronic inflammatory cell infiltration and hyaline degeneration in the fascia. Increased signal intensity in the fascia and tendon sheath was shown on magnetic resonance imaging. In laboratory examination, mild eosinophilia was found in both cases. Both patients had a history of physical activity (weight training and excessive housework, respectively) and showed marked improvement with high doses of oral prednisolone for several months.

Eosinophilic fasciitis: A case report and literature review.

Eosinophilic fasciitis (EF) is a rare scleroderma-like syndrome. There has been no previous published report of EF in a Thai patient. We described a 41 year-old Thai man who presented with symmetric induration of the skin of forearms, arms, hands, fingers, lower aspects of the legs, and feet. Physical examination revealed bilateral symmetrical woody induration of the skin with peau d’orange appearance. A groove sign was positive on the flexural surface of both arms. Laboratory testing revealed a peripheral eosinophil count of 54%. The skin and superficial fascia biopsy specimen from the inner aspect of the left forearm was consistent with EF. He was treated with prednisolone, methotrexate, and colchicine. He experienced a gradual improvement within 4 months. A history of acute onset of scleroderma-like syndrome and careful physical examination can lead us to the diagnosis of EF.

Eosinophilic Fasciitis and Musculoskeletal Complication in a Child : A case report

Eosinophilic fasciitis (EF) in childhood is a very rare, and it is characterized by diffuse fasciitis and peripheral eosinophilia. It has been reported that EF shows good recovery after systemic steroids. We report a case of 18 months old boy with eosinophilic fasciitis which affected the right lower extremity and flank. The case we present here differs from other published reports, in that the patient with eosinophilic fasciitis presented serious musculoskeletal complications such as muscle atrophy, osteoporosis, fracture, and inequality of limb length that were associated with chronic course of steroids therapy. Interdisciplinary approach is critical in preventing complications in children with eosinophilic fasciitis.

Eosinophilic Fasciitis Localized on the Left Lower Extremity: A case report

Eosinophilic fasciitis is a rare disorder which can markedly affect the quality of life in individual patients by disabling joint mobility. Eosinophilic fasciitis causes symmetrical pain, swelling and contracture of the extremities. A definite diagnosis of eosinophilic fasciitis is usually based on histopathologic examination. Hematologically, patients with this disease often have hypergammaglobulinemia and eosinophilia. However, these abnormalities are not specific to this disease and do not correlate with the severity of eosinophilic fasciitis. Although this disease shows good response to systemic steroids, no generally accepted and effective treatment modality has been available. Physical treatment of contracture includes passive, active assistive and active ROM, depending on the condition of the patient, usually after the application of deep or superficial heat. Therapeutic ultrasound is commonly used for contracture. We encountered a case of rehabilitation for an eosinophilic fasciitis localized on the left lower extremity.

Eosinophilic fasciitis preceding relapse of peripheral T-cell lymphoma

Although eosinophilic fasciitis (EF) may precede hematologic malignancy or Hodgkin's disease, association with peripheral T-cell lymphoma (PTCL) is extremely rare. Only four cases of EF preceding or concomitant PTCL have been reported in the world literature. We experienced the first Korean case of EF complicated by the later relapse of peripheral T-cell lymphoma. A 63-year-old Korean male has been followed at our outpatient clinic periodically after treatment for stage IV PTCL. He had been in complete remission for seven and a half years when he developed edema of both lower extremities followed by sclerodermatous skin change in both hands with peripheral eosinophilia. Biopsy from the left hand showed fibrous thickening of the fascia with lymphoplasmacytic and eosinophilic infiltrate, consistent with EF. Twenty-five months later, a newly developed lymph node from the left neck showed recurrence of PTCL. EF may occur as a paraneoplastic syndrome associated with the relapse of PTCL. Therefore, in a patient with EF, the possibility of coexisting and/or future occurrence of hematologic neoplasm should be considered.

A Case of Eosinophilic Fasciitis

Eosinophilic faciitis is a recently recognized entity causing inflammation, thickening and fibrosis of the fascia, which is associated with hypergammaglobulinemia, peripheral eosinolhila. This case of 11 year-old male patient who complaint migrating arthralgia and flexion contration with nodule on left upper extremity for 6 month and suddenly developed proptosis had hypergammaglobulinemia and elevated ESR. Biopsy of the nodule demonstrated inflammatory infiltration of eosinophil in fascia and subcutis, which was consisted with eoainophilic fasciitis. We report a case of eosinophilic fasciitis with brief review who was treated with corticosteroid and had symptomatic improvement.

A Case of Eosinophilic Fasciitis / 대한피부과학회지

We report a case of eosinophilic fasciitis occurring in a 48-year-old man who showed tender, edemstous, indurated, and tight skin on the left forearm and elbow joint. Laboratory findings showed peripheral blood eosinophilia and hypergammaglobulinemia. Other laboratory tests were negative or within normal limits including ANA and anti-DNA. Histopathological findings revealed sclerosis of dermis and thickening of fat and fascia with intense infiltrations of lymphocytes, histiocytes and eosinophils. He was treated successfully with oral prednisolone for 2 weeks.

Dermatoses that Present with Cutaneous Sclerosis

The diseases which present with cutaneous sclerodermatous changes are scleroderma, eosinophilic fasciitis, mixed connective tissue disease, sclerederma adultorum, scleromyxedema and cutaneous midline mucinosis. This paper reviews the characteristics and differential diagnosis among of the above mentioned diseases.