RESUMO
BACKGROUND: Eosinophilic fasciitis (EF) is a rare subtype of deep morphea with an elevated risk of functional impairment. No treatment algorithm has been established for adults with EF refractory to traditional corticosteroid or immunomodulatory treatments. Research on cutaneous and functional outcomes of alternative therapies, such as intravenous immunoglobulin (IVIG), remains scarce. Objective: To describe the functional and cutaneous outcomes associated with IVIG in adults with treatment-refractory EF at a tertiary referral center. METHODS: We performed a retrospective chart review of 18 consecutive patients with EF identified through a billing code search seen within the UCSF Department of Dermatology between 2015 and 2022. Results: Seven patients (41.2%) underwent at least one course of intravenous immunoglobulins (IVIG) during the study period. Of 6 patients with available follow-up data, 5 patients (83.3%) achieved both sustained cutaneous and functional improvement. In the IVIG cohort, 1 patient (16.7%) achieved complete response with relapse, 4 (66.7%) were partial responders, and 1 (16.7%) was a non-responder who required treatment with mepolizumab. CONCLUSION: Adverse effects of IVIG included headaches in 1 patient (14.3%) and rash in 2 patients (28.6%). There were no reported veno-occlusive or thromboembolic events associated with IVIG. J Drugs Dermatol. 2024;23(4):8017. doi:10.36849/JDD.8017e.
Assuntos
Eosinofilia , Fasciite , Imunoglobulinas Intravenosas , Adulto , Humanos , Imunoglobulinas Intravenosas/efeitos adversos , Estudos Retrospectivos , Resultado do Tratamento , Fasciite/diagnóstico , Fasciite/tratamento farmacológico , Fasciite/induzido quimicamenteRESUMO
We report a case of eosinophilic fasciitis in a teenage auto mechanic who was most likely affected by occupational exposure to organic solvents, including the aromatic hydrocarbons benzene, trimethylbenzene, naphthalene, toluene, and xylene. The patient presented with an 8-month history of painful induration of his extremities and an abnormal gait. A deep excisional biopsy of the fascia was obtained, demonstrating subcutaneous fibrosis with perivascular and interstitial inflammation, with lymphocytes and plasma cells spilling into the sclerosed fascia, and focal fibrinoid necrosis. Treatment was begun with intravenous pulse doses of methylprednisolone, prednisone (20 mg daily), and subcutaneous methotrexate (25 mg weekly), and the patient's painful induration had resolved and gait had normalized at the 6-month follow-up. Our case suggests that exposure to organic solvents could be implicated in the pathogenesis of eosinophilic fasciitis and highlights the importance of a thorough occupational history to prevent repeat exposures to potentially causative agents.
Assuntos
Eosinofilia , Fasciite , Adolescente , Humanos , Masculino , Fasciite/induzido quimicamente , Eosinofilia/induzido quimicamente , Tolueno , SolventesRESUMO
PURPOSE: Below knee amputation (BKA) is a common surgical procedure for diabetic foot ulcers and necrotizing lower limb fasciitis patients. However, it is a painful procedure and inadequate postoperative analgesia impedes rehabilitation and prolongs hospitalization. An ideal pain management regimen should provide superior analgesia while minimizing opioid consumption and improving rehabilitation. METHODS: We retrospectively reviewed medical charts of 218 patients who underwent BKA for diabetic foot ulcer or necrotizing lower limb fasciitis at a single center between January 2017 and September 2020. Two groups were analyzed: patients who received dual nerve block (DNB) before surgery (Group I; n = 104), and patients who did not (Group II; n = 93). By the exclusion criteria, 21 patients were excluded. The femoral and sciatic nerves were each blocked separately under ultrasound guidance. This procedure was performed immediately before the operation. RESULTS: Group I patients' subjective pain scores were significantly lower than that of Group II at 6, 12, and 24 h after BKA (P < 0.05). Group I's morphine milligram equivalent (MME) was significantly lower than those of Group II at 72 h after BKA (P < 0.05). Moreover, the rate of postoperative nausea and vomiting (PONV) and delirium was significantly lower in Group I patients than that in Group II patients. CONCLUSION: Ultrasound-guided lower extremity nerve block surgery is excellent for early postoperative pain control, could be used as an accurate and effective pain control method, and can reduce the side effects of opioid consumption after BKA.
Assuntos
Artroplastia do Joelho , Pé Diabético , Fasciite , Bloqueio Nervoso , Humanos , Analgésicos Opioides/uso terapêutico , Analgésicos Opioides/farmacologia , Dor Pós-Operatória/tratamento farmacológico , Estudos Retrospectivos , Nervo Femoral , Artroplastia do Joelho/métodos , Bloqueio Nervoso/métodos , Ultrassonografia de Intervenção , Amputação Cirúrgica , Fasciite/induzido quimicamente , Fasciite/tratamento farmacológico , Anestésicos Locais/efeitos adversosRESUMO
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 0.07×109/L to 3.3×109/L. Magnetic resonance imaging showed thickened skin of both forearms, while T2-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 0.17×109/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.
Assuntos
Eosinofilia , Fasciite , Dermatopatias , Masculino , Humanos , Pessoa de Meia-Idade , Inibidores de Checkpoint Imunológico/uso terapêutico , Eosinofilia/diagnóstico , Eosinofilia/tratamento farmacológico , Eosinofilia/patologia , Fasciite/induzido quimicamente , Mialgia , Dermatopatias/complicações , Dermatopatias/tratamento farmacológicoRESUMO
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.
Assuntos
Doenças Autoimunes , Fasciite , Doenças Autoimunes/tratamento farmacológico , Fasciite/induzido quimicamente , Fasciite/tratamento farmacológico , Glucocorticoides/uso terapêutico , Humanos , Imunossupressores/efeitos adversos , Ácido Micofenólico/efeitos adversos , Ácido Micofenólico/uso terapêuticoAssuntos
Antineoplásicos Imunológicos/efeitos adversos , Eosinofilia/induzido quimicamente , Fasciite/induzido quimicamente , Inibidores de Checkpoint Imunológico/efeitos adversos , Melanoma/tratamento farmacológico , Nivolumabe/efeitos adversos , Esclerodermia Localizada/induzido quimicamente , Quimioterapia Adjuvante , Eosinofilia/patologia , Fasciite/patologia , Humanos , Masculino , Melanoma/cirurgia , Pessoa de Meia-Idade , Esclerodermia Localizada/patologiaAssuntos
Anticorpos Monoclonais Humanizados/efeitos adversos , Carcinoma Hepatocelular/complicações , Fasciite/induzido quimicamente , Neoplasias Hepáticas/complicações , Carcinoma Hepatocelular/tratamento farmacológico , Humanos , Neoplasias Hepáticas/tratamento farmacológico , Masculino , Pessoa de Meia-IdadeAssuntos
Eosinofilia/induzido quimicamente , Fasciite/induzido quimicamente , Fatores Imunológicos/efeitos adversos , Natalizumab/efeitos adversos , Eosinofilia/diagnóstico por imagem , Eosinofilia/patologia , Fasciite/diagnóstico por imagem , Fasciite/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla/tratamento farmacológico , UltrassonografiaAssuntos
Dermatomiosite/epidemiologia , Eosinofilia/epidemiologia , Fasciite/epidemiologia , Inibidores de Checkpoint Imunológico/efeitos adversos , Lúpus Eritematoso Cutâneo/epidemiologia , Esclerodermia Localizada/epidemiologia , Idoso , Anticorpos Antinucleares/sangue , Anticorpos Antinucleares/imunologia , Dermatomiosite/sangue , Dermatomiosite/induzido quimicamente , Dermatomiosite/imunologia , Eosinofilia/sangue , Eosinofilia/induzido quimicamente , Eosinofilia/imunologia , Fasciite/sangue , Fasciite/induzido quimicamente , Fasciite/imunologia , Feminino , Humanos , Lúpus Eritematoso Cutâneo/sangue , Lúpus Eritematoso Cutâneo/induzido quimicamente , Lúpus Eritematoso Cutâneo/imunologia , Masculino , Pessoa de Meia-Idade , Neoplasias/tratamento farmacológico , Neoplasias/imunologia , Estudos Retrospectivos , Esclerodermia Localizada/sangue , Esclerodermia Localizada/induzido quimicamente , Esclerodermia Localizada/imunologiaRESUMO
Aluminium hydroxide is a well-known adjuvant used in vaccines. Although it can enhance an adaptive immune response to a co-administered antigen, it causes adverse effects, including macrophagic myofasciitis (MMF), subcutaneous pseudolymphoma, and drug hypersensitivity. The object of this study is to demonstrate pediatric cases of aluminium hydroxide-induced diseases focusing on its rarity, under-recognition, and distinctive pathology. Seven child patients with biopsy-proven MMF were retrieved from the Seoul National University Hospital (SNUH) pathology archives from 2015 to 2019. The medical records and immunisation history were reviewed, and a full pathological muscle examination was carried out. The mean age was 1.7 years (8.9-40 months), who had records of vaccination against hepatitis B, hepatitis A, and tetanus toxoid on the quadriceps muscle. The chief complaints were muscle weakness (n = 6), delayed motor milestones (n = 6), instability, dysarthria, and involuntary movement (n = 1), swallowing difficulty (n = 1), high myopia (n = 1), and palpable subcutaneous nodules with skin papules (n = 1). Muscle biopsy showed MMF (n = 6) and pseudolymphoma (n = 1) with pathognomic basophilic large macrophage infiltration, which had distinctive spiculated inclusions on electron microscopy. The intracytoplasmic aluminium was positive for PAS and Morin stains. Distinctive pathology and ultrastructure suggested an association with aluminium hydroxide-containing vaccines. To avoid misdiagnosis and mistreatment, we must further investigate this uncommon condition, and pharmaceutical companies should attempt to formulate better adjuvants that do not cause such adverse effects.
Assuntos
Adjuvantes Imunológicos/efeitos adversos , Hidróxido de Alumínio/efeitos adversos , Hipersensibilidade a Drogas/etiologia , Fasciite/induzido quimicamente , Miosite/induzido quimicamente , Pseudolinfoma/induzido quimicamente , Vacinação/efeitos adversos , Vacinas Virais/efeitos adversos , Pré-Escolar , Hipersensibilidade a Drogas/diagnóstico , Hipersensibilidade a Drogas/imunologia , Fasciite/diagnóstico , Fasciite/imunologia , Feminino , Hepatite A/imunologia , Hepatite A/prevenção & controle , Hepatite A/virologia , Hepatite B/imunologia , Hepatite B/prevenção & controle , Hepatite B/virologia , Humanos , Lactente , Macrófagos/efeitos dos fármacos , Macrófagos/imunologia , Macrófagos/patologia , Masculino , Debilidade Muscular/induzido quimicamente , Debilidade Muscular/diagnóstico , Debilidade Muscular/imunologia , Miosite/diagnóstico , Miosite/imunologia , Pseudolinfoma/diagnóstico , Pseudolinfoma/imunologia , Tela Subcutânea , Tétano/imunologia , Tétano/prevenção & controle , Tétano/virologia , Vacinas Virais/administração & dosagemRESUMO
OBJECTIVE: To assess: (i) the prevalence, and clinical and imaging characteristics of immune checkpoint inhibitor (ICI)-induced musculoskeletal immune-related adverse events (ir-AEs) in a prospective manner and (ii) whether serum levels of cytokines associated with the Th1/Th2/Th17 response are differentially expressed in patients with and without musculoskeletal Ir-AEs. METHODS: All patients treated with ICI who developed musculoskeletal manifestations were referred to the Rheumatology Department, and an MRI of the involved area(s) was performed. RESULTS: During the study period, a total of 130 patients were treated with ICIs. Of these, 10 (7.7%) developed ICI-induced Ir-AEs. The median time from ICI treatment since development of symptoms was 2.5 months. Three different patterns of musculoskeletal manifestations were found: (i) prominent joint involvement (n = 3); (ii) prominent 'periarticular' involvement (n = 4). These patients had diffuse swelling of the hands, feet or knees. MRI depicted mild synovitis with more prominent myositis and/or fasciitis in the surrounding tissues in all cases; (iii) myofasciitis (n = 3). Clinically, these patients presented with pain in the knee(s)/thigh(s), whereas MRI depicted myofasciitis of the surrounding muscles. Patients with musculoskeletal ir-AEs had significantly higher oncologic response rates compared with patients not exhibiting musculoskeletal ir-AEs (50% vs 12.5%, respectively, P = 0.0016). Cytokine levels associated with a Th1/Th2/Th17 response were similar between patients with and without musculoskeletal ir-AEs. Overall, symptoms were mild/moderate and responded well to treatment, with no need for ICI discontinuation. CONCLUSION: In our cohort, ICI-induced musculoskeletal manifestations developed in 7.7% of patients. Imaging evidence of myofasciitis was found in most patients, indicating that the muscle/fascia is more frequently involved than the synovium.
Assuntos
Antineoplásicos Imunológicos/efeitos adversos , Fatores Imunológicos/efeitos adversos , Imageamento por Ressonância Magnética/métodos , Doenças Musculoesqueléticas/induzido quimicamente , Doenças Reumáticas/induzido quimicamente , Antineoplásicos Imunológicos/administração & dosagem , Estudos de Coortes , Citocinas/metabolismo , Relação Dose-Resposta a Droga , Esquema de Medicação , Fasciite/induzido quimicamente , Fasciite/diagnóstico por imagem , Fasciite/epidemiologia , Feminino , Humanos , Fatores Imunológicos/administração & dosagem , Incidência , Masculino , Pessoa de Meia-Idade , Doenças Musculoesqueléticas/diagnóstico por imagem , Doenças Musculoesqueléticas/epidemiologia , Miosite/induzido quimicamente , Miosite/diagnóstico por imagem , Miosite/epidemiologia , Estudos Prospectivos , Doenças Reumáticas/diagnóstico por imagem , Doenças Reumáticas/epidemiologia , Índice de Gravidade de DoençaRESUMO
BACKGROUND: Checkpoint inhibitor therapy is widely known to cause a number of immune-related adverse events. One rare adverse effect that is emerging is eosinophilic fasciitis, a fibrosing disorder causing inflammatory infiltration of subcutaneous fascia. It is characterized clinically by edema and subsequent induration and tightening of the skin and subcutaneous tissues. The condition is rare, yet at our institutions we have seen four cases in the past 3 years. We describe our 4 cases and review 11 other cases reported in the literature. CASE PRESENTATION: We present four cases of eosinophilic fasciitis following treatment with programmed cell death protein 1 or programmed cell death-ligand 1 blockade. All patients had extremity involvement with characteristic skin changes ranging from peripheral edema to induration, tightening, and joint limitation. The patients had varying degrees of peripheral eosinophilia. In two of our patients, the diagnosis was made by full-thickness skin biopsy showing lymphocytic infiltration of the subcutaneous fascia, with CD4+ T cells predominating in one case and CD8+ T cells in the other. In the other two cases, the diagnosis was made on the basis of characteristic imaging findings in the context of clinical features consistent with the diagnosis. All four patients were treated with glucocorticoids with varying degrees of success; immunotherapy had to be discontinued in all four. Patients with advanced melanoma who experienced this adverse effect had either a partial response or a complete response to therapy. CONCLUSION: Eosinophilic fasciitis can occur as a result of checkpoint inhibitor therapy. Although a tissue diagnosis is the gold standard, imaging studies may facilitate the diagnosis in the presence of consistent clinical features, but a degree of suspicion is key to recognizing the condition early. Therapy requires a collaborative approach by oncology, rheumatology, and dermatology; physical therapy is an important adjunct in treatment. For advanced melanoma, it may be a good prognostic indicator. IMPLICATIONS FOR PRACTICE: It is important for clinicians to recognize that eosinophilic fasciitis is a potential immune-related adverse event (irAE) as a consequence of immune checkpoint inhibitor therapy. The presentation is quite stereotypical; the diagnosis can be made by imaging in the absence of a full-thickness skin biopsy. Early intervention is important to limit morbidity. This irAE may be a good prognostic sign among patients with melanoma.
Assuntos
Eosinofilia , Fasciite , Edema , Eosinofilia/induzido quimicamente , Fasciite/induzido quimicamente , Glucocorticoides , HumanosRESUMO
INTRODUCTION: Chronic enterovirus infections can occur in primary immunodeficiency with hypogammaglobulinemia. They usually associate meningitis and myofasciitis. Such infections have also been described in adults with rituximab-induced hypogammaglobulinemia. CASE REPORT: We report the case of a 33-year-old woman who was given rituximab for immune thrombocytopenia and developed rituximab-induced hypogammaglobulinemia (IgG 4.4g/L). One year after the last rituximab infusion, she developed lower limbs myofasciitis, followed two months later by a chronic lymphocytic meningitis. PCR in the serum and the cerebrospinal fluid at the time of the meningitis and the myofasciitis were positive to the same enterovirus (echovirus 11) while it was negative in the fascia biopsy. Under treatment with intravenous immunoglobulins, all symptoms and laboratory abnormalities improved and enterovirus PCR became negative. CONCLUSION: We report a case of chronic enterovirus infection associating meningitis and myofasciitis in an adult with rituximab-induced hypogammaglobulinemia. Outcome was favorable under treatment with intravenous immunoglobulins.