Eosinophilic granuloma of the clavicle in an 11-year-old Chinese girl: A case report.

RATIONALE: Eosinophilic granuloma (EG) - the most common form of Langerhans cell histiocytosis - occurs rarely, and manifestations with only rib and clavicle involvement are extremely rare. EG symptoms often include pain, swelling, and soft tissue mass. The clinical diagnosis of bone EG is complex, and the differential diagnosis includes Ewing sarcoma, tuberculosis, multiple myeloma, lymphoma, primary bone malignancy, and other osteolytic lesions. PATIENTS CONCERN: The patient was an 11-year-old female who found a subcutaneous mass at the junction of the right clavicle and sternum 2 days before presenting at the clinic without apparent triggers. Initially, we considered a subcutaneous cyst or inflammatory mass. Color ultrasound and computed tomography examination revealed osteomyelitis. Finally, the patient was diagnosed with EG after a pathological tissue biopsy, and the child recovered after surgery and anti-infective treatment. DIAGNOSIS: The patient underwent surgery to remove the tumor at a specialist hospital and was diagnosed with EG by pathological examination. INTERVENTION: The patient went to a specialist hospital for surgery to remove the mass and underwent anti-infective treatment. OUTCOMES: The patient recovered after surgical resection and antibiotic treatment. LESSONS: In this report, we emphasize that the clinical presentation of EG in children is not specific. Furthermore, examining age, history, presence of symptoms, and the number of sites is essential to make a correct diagnosis, and a histological examination is necessary to confirm the diagnosis.

Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature.

INTRODUCTION: Eosinophilic granuloma (EG) is the most common form of Langerhans cell histiocytosis, presenting as a single osteolytic lesion of the calvarium. Its diagnosis is based on typical clinical and radiological features. While surgical resection has been the standard treatment for EG, growing evidence favors watchful waiting, as unifocal calvarial lesions appear to frequently undergo spontaneous remission. However, histopathological confirmations of this hypothesis are still very limited. METHODS: Methods. Here, we report a case of EG with typical clinical and radiological features which, due to intervening circumstances, was resected in a delayed fashion. Moreover, we perform a systematic review of the literature on conservative management of EG. RESULTS: In our case, histological examination showed ongoing bone regeneration with no traces of the disease. Through our literature review, we found 47 cases of calvarial EG managed with watchful waiting. No active intervention was required in 43 cases (91%). Four patients (9%) received surgery or chemotherapy due to the persistence/progression of symptoms or family request. Three reports other than ours documented spontaneous disease remission in surgically resected EG upon histopathological examination. CONCLUSION: Our report provides further evidence that watchful waiting can be a reasonable option in the management of single calvarial EG.

Acquired butterfly vertebra as a sequela of eosinophilic granuloma.

Butterfly vertebras are an abnormal embryological formation of the spinal bodies that occur because of a lack of fusion of the chondrification centers of the vertebral bodies. Langerhans cell histiocytosis is an entity that frequently involves vertebral bodies resulting in flat vertebras, and recovery of the vertebral body height is a very unusual finding. We present a case report of a pediatric patient with a thoracic acquired butterfly vertebra which occurred secondary to a Langerhans cell histiocytosis involvement. It is extremely rare to find vertebra plana that regains its complete height but is even more infrequent to evidence of a butterfly vertebra deformity that is not congenital.

Granuloma eosinofílico cervical en el adolescente: reporte de un caso y revisión de la literatura / Cervical eosinophilic granuloma in adolescents: case report and literature review

INTRODUCCIÓN El granuloma eosinofílico (GE) es una patología infrecuente, sobre todo en adultos, que puede afectar la columna cervical. A pesar de la vasta literatura, esta enfermedad afecta principalmente a la población infantil, y no hay un consenso sobre el manejo en adultos. Con el objetivo de aportar conocimiento respecto a esta patología poco frecuente, se presenta un caso clínico de GE cervical en un paciente de 16 años, a quien se trató de manera conservadora, con buenos resultados y retorno completo a sus actividades. CASO CLÍNICO Un hombre de 16 años, seleccionado de rugby, consultó por dolor cervical axial persistente y nocturno de 6 semanas de evolución, sin trauma evidente. Al examen, destacó dolor a la compresión axial sin compromiso neurológico asociado. Los exámenes de tomografía computarizada (TC) y resonancia magnética (RM) revelaron lesión lítica en el cuerpo de C3 de características agresivas, de presentación monostótica en tomografía por emisión de positrones-tomografía computada (TEP-TC) compatible con tumor primario vertebral. Se decidió realizar biopsia percutánea bajo TC, para definir el diagnóstico y manejo adecuado, la cual fue compatible con células de Langerhans. Al no presentar clínica ni imagenología de inestabilidad ósea evidente o compromiso neurológico, se manejó con tratamiento conservador, inmovilización cervical, analgesia oral, y seguimiento estrecho. A los cuatro meses de evolución, se presentó con una TC con cambios reparativos del cuerpo vertebral y sin dolor, y logró retomar sus actividad habituales. CONCLUSIONES El diagnóstico de GE es infrecuente a esta edad, y se debe plantear entre diagnósticos diferenciales de lesiones líticas agresivas primarias vertebrales. Es necesario el uso de imágenes, y la biopsia vertebral es fundamental para confirmar el diagnóstico. Su manejo va a depender de la sintomatología, del compromiso de estructuras vecinas, y de la estabilidad de la vértebra afectada. El manejo conservador con seguimiento clínico e imagenológico es una opción viable.

INTRODUCTION Eosinophilic granuloma (EG) is a rare, tumor-like lesion, infrequently affecting the cervical spine, particularly in adults. Although vastly described in literature, this pathology mainly affects children, and there is still no consensus on its treatment in older patients. With the goal of contributing to increase the knowledge regarding this infrequent pathology, we present a case of a C3 eosinophilic granuloma in a 16-year-old patient, who was treated conservatively, with good results, including complete return to his previous activities. CLINICAL CASE a 16-year-old male, elite rugby player, presented with a history of persistent neck pain, mainly at night, with no previous trauma. Upon physical examination, he reported neck pain with axial compression of the head, without neurological impairment. Both computed tomography (CT) and magnetic resonance imaging (MRI) scan revealed an aggressive lytic lesion in the C3 vertebral body, a with monostotic presentation on positron emission tomography-computed tomography (PET-CT) compatible with a primary spine tumor. A CT-guided percutaneous biopsy was obtained to establish the diagnosis and provide the proper management. The results were compatible with Langerhans cells. As he presented no symptoms or imaging findings of evident bone instability, as well as no neurological impairment, the patient was treated conservatively, with a cervical brace, oral pain medication and close followup. A CT obtained after four months of treatment showed reparative changes of the C3 vertebral body; at this point, the patient reported no neck pain, so he was able to return to his previous activities. CONCLUSIONS Although an EG is rare at this age, it should be considered in the differential diagnosis of primary vertebral aggressive lytic lesions. Imaging and a vertebral biopsy are paramount to confirm the diagnosis. The treatment modality depends on the symptoms, the involvement of adjacent structures, and the stability of the affected vertebra. Conservative management including clinical and imaging followup is a viable option.

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis.

OBJECTIVE: To develop and validate revised classification criteria for eosinophilic granulomatosis with polyangiitis (EGPA). METHODS: Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in five phases: (1) identification of candidate criteria items using consensus methodology, (2) prospective collection of candidate items present at the time of diagnosis, (3) data-driven reduction of the number of candidate items, (4) expert panel review of cases to define the reference diagnosis and (5) derivation of a points-based risk score for disease classification in a development set using least absolute shrinkage and selection operator logistic regression, with subsequent validation of performance characteristics in an independent set of cases and comparators. RESULTS: The development set for EGPA consisted of 107 cases of EGPA and 450 comparators. The validation set consisted of an additional 119 cases of EGPA and 437 comparators. From 91 candidate items, regression analysis identified 11 items for EPGA, 7 of which were retained. The final criteria and their weights were as follows: maximum eosinophil count ≥1×109/L (+5), obstructive airway disease (+3), nasal polyps (+3), cytoplasmic antineutrophil cytoplasmic antibody (ANCA) or anti-proteinase 3-ANCA positivity (-3), extravascular eosinophilic predominant inflammation (+2), mononeuritis multiplex/motor neuropathy not due to radiculopathy (+1) and haematuria (-1). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as having EGPA if the cumulative score was ≥6 points. When these criteria were tested in the validation data set, the sensitivity was 85% (95% CI 77% to 91%) and the specificity was 99% (95% CI 98% to 100%). CONCLUSION: The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis demonstrate strong performance characteristics and are validated for use in research.

Eosinophilic Granuloma of the Liver Mimicking Metastatic Liver Tumor.

We herein report a case of coagulation necrosis with granulation and eosinophilic infiltration of the liver. A 37-year-old woman was diagnosed with a new mass lesion in the liver 1 month after breast cancer surgery and admitted for a further examination. Because the tumor occurred immediately after surgery, it was considered essential to determine whether or not it was a metastatic liver tumor from breast cancer. A percutaneous liver tumor biopsy revealed eosinophilic granuloma of the liver, which is considered to have a high possibility of visceral larva migrans with suspected gnathostomiasis infection. A detailed medical history and histological diagnosis are important for making a differential diagnosis.

Unique Recognizable Histopathologic Variant of Palisaded Neutrophilic and Granulomatous Dermatitis that Is Associated With SRSF2-Mutated Chronic Myelomonocytic Leukemia: Case Report and Review of the Literature.

ABSTRACT: Palisaded neutrophilic and granulomatous dermatitis (PNGD) represents a cutaneous histopathologic reaction spectrum associated with several underlying disorders. Few cases of PNGD have been associated with chronic myelomonocytic leukemia (CMML), a malignant hematopoietic disorder with features in between those of a myeloproliferative neoplasm and myelodysplastic syndrome. We present a patient with a generalized papular skin reaction involving the neck, chest, and shoulders with histomorphological features on the spectrum of PNGD. Subsequent laboratory workup demonstrated a persistent mild monocytosis, raising concern for CMML. The diagnosis was ultimately confirmed with a bone marrow biopsy and associated mutational analysis through next-generation sequencing which identified deleterious variants in SRSF2, IDH2, and ASXL1. The findings in this case strengthen the previously made association between PNGD and SRSF2-mutated CMML and may help better define a unique recognizable clinical-histopathological-molecular subtype for dermatopathologists.

IgG anti-Pentraxin 3 antibodies are a novel biomarker of ANCA-associated vasculitis and better identify patients with eosinophilic granulomatosis with polyangiitis.

OBJECTIVE: To investigate prevalence of anti-Pentraxin 3 (PTX3) antibodies in sera of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) patients. METHODS: Anti-PTX3 and PTX3 levels were analysed by enzyme-linked immunosorbent assays in sera from unselected patients with AAV and compared with patients with systemic lupus erythematosus (SLE, n = 130), other connective tissue diseases (CTDs, n = 97) and matched healthy controls (n = 97). Optical density (OD) cut-off for positive anti-PTX3 antibodies was determined by ROC curve analysis and set as 0.234. Indirect immunofluorescence (IIF) on fixed human granulocytes was used to analyze the fluorescence pattern of anti-PTX3 antibodies. Liquid-phase inhibition tests were conducted to assess potential interferences. RESULTS: We included 101 AAV patients (females 58%, median age 60[51-69] years) affected either with granulomatosis with polyangiitis (GPA, n = 51), microscopic polyangiitis (MPA, n = 12) or eosinophilic granulomatosis with polyangiitis (EGPA, n = 38). Anti-PTX3 antibodies were detected in 29.7% AAV patients, being significantly higher than in healthy controls (p < 0.001) and CTDs (p = 0.030) but lower than in SLE (p = 0.004). Anti-PTX3 antibody prevalence was 44.7% in EGPA, 25% in MPA and 19% in GPA (p = 0.034). Among ANCA negative patients, 35.7% displayed positive anti-PTX3 antibodies. Anti-PTX3 were associated with a lower prevalence of systemic (p = 0.002), ear-nose-throat (p = 0.006) and renal manifestations (p = 0.016). Anti-PTX3 antibodies were characterized by a specific IIF pattern on fixed granulocytes. PTX3 serum levels resulted lower in AAV than healthy controls (p < 0.001). PTX3 inhibited anti-PTX3 binding in a dose-dependent manner. CONCLUSIONS: Anti-PTX3 autoantibodies appear a promising novel biomarker of AAV, especially EGPA.

Multifocal eosinophilic granuloma of the jaws with long-term follow-up: a case report.

Eosinophilic granuloma, a rare disease, has various clinical manifestations and no specific X-rays features and is thus easily misdiagnosed. This paper reports a case of multifocal eosinophilic granuloma of jaw with long-term follow-up. The patient initially presented with periodontal tissue destruction.The diagnosis, treatment and prognosis of multifocal eosinophilic granuloma of jaw were discussed in combination with the literature to alert this disease in clinical practice.

Recurrent eosinophilic granuloma involving maxilla and mandible in an adult male: an unusual case report.

Langerhans' cell histiocytosis (LCH) is a disease spectrum characterized by the intense infiltration/accumulation of Langerhans' cells in the affected tissues. Eosinophilic granuloma is the mildest form of LCH which mainly affects the skull, mandible, ribs and femur in children and young adults but rarely occurs in the maxilla. Here, we describe an unusual case of eosinophilic granuloma affecting the tissues adjacent to tooth #25 and tooth #46 (FDI numbering system) in a 35-year-old male. Although lesions underwent clinical remission after local radiotherapy, a new lesion appeared 11 months after initial therapy in tooth #18, indicating recurrence of eosinophilic granuloma. Subsequently, the patient was treated with corticosteroid therapy, achieved remission and is disease free after 3 years of follow-up.

Traumatic ulcerative granuloma with stromal eosinophilia, an unusual clinical manifestation in the oral mucosa: case report / Granuloma ulcerativo traumático con eosinofilia estromal, una manifestación clínica inusual en la mucosa oral: reporte de un caso

Introduction: Traumatic ulcerative granuloma with stromal eosinophilia is an uncommon condition of the oral mucosa with a chronic course, usually affecting the tongue. Case Report: Clinically it presents as a chronic ulcer, with raised and indurated borders, rarely presented as a tumor. Histologically it shows a diffuse mixed inflammatory infiltrate, rich in eosinophils. The etiology of this lesion is still unclear; however, chronic irritation from traumatic agents is considered a major initiating factor. In some cases, the presence of CD30+ mononuclear cells within the lesions suggest the possibility of a CD30+ lymphoproliferative disorder. This article presents a case of a traumatic ulcerative granuloma with stromal eosinophilia manifested in a 56-year-old female with a solitary ulcerated tumor inside the right cheek. Conclusion: It was diagnosed based on clinical data and histopathological features. In a brief literature review, the entity has been characterized, analyzing its etiology and nature.

Introducción: El granuloma ulcerativo traumático con eosinofilia estromal es una afección infrecuente de la mucosa oral de curso crónico, que suele afectar a la lengua. Case Report: Clínicamente se presenta como una úlcera crónica, con bordes elevados e indurados, rara vez se presenta como un tumor. Histológicamente muestra un infiltrado inflamatorio mixto difuso, rico en eosinófilos. La etiología de esta lesión aún no está clara; sin embargo, la irritación crónica por agentes traumáticos se considera un factor de iniciación importante. En algunos casos, la presencia de células mononucleares CD30 + dentro de las lesiones sugiere la posibilidad de un trastorno linfoproliferativo CD30+. En este artículo se presenta el caso de un granuloma ulcerativo traumático con eosinofilia estromal que se manifiesta en una mujer de 56 años con un tumor ulcerado solitario en el interior de la mejilla derecha. Conclusión: Se diagnosticó con base en datos clínicos y características histopatológicas. En una breve revisión de la literatura se ha caracterizado la entidad, analizando su etiología y naturaleza.

Eosinophilic granuloma of cranial location: report of a case and bibliographic review / [Granuloma eosinófilo de localización craneal: reporte de un caso y revisión bibliográfica]

Introduction: Eosinophilic granuloma is an unusual benign disease that usually affects the pediatric population and young adults. It is the most benign of the diseases traditionally known as histiocytosis X that are now called Langerhans cell histiocytosis. Clinical Case: Pediatric patient with a painful lump in the temporal region. The imaging tests carried out reveal the existence of an osteolytic lesion with an aggressive pattern compatible with eosinophilic granuloma. The patient underwent surgery with a conclusive definitive histological diagnosis of eosinophilic granuloma. Discussion: Eosinophilic granuloma can affect one or multiple bones, of which the most frequent are the cranial bones, epiphyses of long bones and ribs, requiring individualized diagnosis and treatment strategies for optimal management and results, with surgical treatment of First choice. Conclusion: Eosinophilic granuloma is an infrequent benign condition that requires a correct anamnesis and clinical examination of the patient, as well as the demonstration of the characteristic radiological images, allowing a generally accurate presumptive diagnosis to be reached that in most cases can be considered definitive.

Introducción: El granuloma eosinófilo es una enfermedad benigna poco usual que suele afectar a la población pediátrica y adultos jóvenes. Es la afección más benigna de las enfermedades tradicionalmente conocidas como histiocitosis X que en la actualidad reciben la denominación de histiocitosis de células de Langerhans. Caso Clínico: Paciente pediátrico con bultoma doloroso en región temporal. Las pruebas de imagen realizadas ponen de manifiesto la existencia de una lesión osteolítica con patrón de agresividad compatible con granuloma eosinófilo. El paciente es intervenido mediante cirugía con diagnóstico histológico definitivo concluyente de granuloma eosinófilo. Discusión: El granuloma eosinófilo puede afectar a uno o múltiples huesos, de los cuáles los más frecuentes son los huesos craneales, epífisis de huesos largos y costillas, precisando estrategias de diagnóstico y tratamiento individualizadas para un manejo y resultado óptimo, siendo el tratamiento quirúrgico de primera elección. Conclusión: El granuloma eosinófilo es un cuadro benigno infrecuente que requiere de una correcta anamnesis y exploración clínica del paciente, así como la demostración de las imágenes radiológicas características, permitiendo llegar a un diagnóstico de presunción generalmente certero que en la mayoría de ocasiones se puede considerar definitivo.

Histopathologic reaction patterns to differentially cross-linked hyaluronic acid fillers: A retrospective case series.

BACKGROUND: Hyaluronic acid filler reactions have been increasingly observed in recent years. Our study investigates whether the increased number of filler reactions observed since 2014 is associated with a specific histopathologic inflammatory pattern and type of filler. METHODS: The institution's dermatopathology electronic database was retrospectively searched for histopathologic reactions to hyaluronic acid from January 2014 to December 2019. The age, sex, type of filler, procedure, location, and histopathologic patterns were recorded. RESULTS: From 2014 to 2019, there were 15 cases of granulomatous reactions to hyaluronic acid filler. In 10 of these cases, there was a characteristic inflammatory pattern characterized by tightly cuffed palisades of histiocytes with varying numbers of eosinophils. Of the 11 cases in which the type of filler was known, all used Vycross technology, a novel manufacturing process in the production of hyaluronic acid filler. CONCLUSION: A characteristic histopathologic pattern of discrete foci of tightly cuffed palisaded granulomas with eosinophils is associated with fillers manufactured using Vycross technology.

Eosinophilic Granulomatosis With Polyangiitis: An Unusual Case of Pediatric Subglottic Stenosis.

Systemic disease is an uncommon cause of subglottic stenosis (SGS). We report a case of severe SGS due to underlying eosinophilic granulomatosis with polyangiitis (EGPA) in a child presenting with isolated stridor. EGPA is a rare systemic vasculitis with very limited cases reported in the pediatric population. While surgical intervention was required given the degree of stenosis in this case, medical management of the underlying systemic disease process is critical when there is clinical suspicion of SGS in the context of systemic vasculitis. Laryngoscope, 131:656-659, 2021.

Cardiac involvement in the adult primary vasculitides.

INTRODUCTION: Heart involvement in vasculitis is rare, but potentially severe. The ascertainment of cardiac disease in vasculitis is complex and requires an integrated multidisciplinary approach involving the Rheumatologist, Radiologist, Cardiologist, and Heart surgeon. AREAS COVERED: the authors searched PubMed using the keywords 'heart'[Mesh] and vasculitis"[Mesh]. EXPERT OPINION: Virtually any vasculitis can affect the heart, but cardiac involvement is more common in some vasculitides such as Takayasu arteritis, polyarteritis nodosa, and eosinophilic granulomatosis with polyangiitis. Immunosuppressive treatment and when indicated surgery can improve the prognosis.

Nasal polyps in eosinophilic granulomatosis with polyangiitis: Structured histopathology and CD105 expression.

PURPOSE: Distinguishing the prodromal nasal polyposis of eosinophilic granulomatosis with polyangiitis (EGPA) from chronic rhinosinusitis with nasal polyps (CRSwNP) is a challenge for rhinologists and rheumatologists. It has recently been reported that angiogenesis and CD105 expressed on vascular endothelial cells could have a role in the pathogenesis and development of nasal polyps. This exploratory study examined the structured histopathology of nasal polyps in patients with EGPA and CRSwNP, comparing CD105 expression in their nasal tissue with that of a control group with no chronic sinonasal inflammation. METHODS: A structured histopathological study was performed on surgical specimens of nasal tissue from 32 adults (13 with EGPA, 14 with CRSwNP, 5 controls), considering CD105 as a marker to determine microvessel density (MVD). RESULTS: The mean eosinophil count was higher in EGPA patients with tissue inflammation (p = .002), and in CRSwNP patients with sub-epithelial edema (p = .009). Neutrophil infiltration was significantly associated with severe tissue inflammation in EGPA patients (p = .04), but with the absence of fibrosis in CRSwNP patients (p = .04). In the EGPA group, CD105-MVD correlated with tissue eosinophil count (p = .05). Mean CD105-MVD was significantly higher in EGPA patients with mucosal ulceration (p = .004). In the CRSwNP group, a CD105-MVD correlated positively and significantly with tissue eosinophil count (p = .01). CONCLUSION: Alongside the known abundance of eosinophils, other cells might contribute to inflammatory processes. Neutrophils may amplify inflammation, eosinophil recruitment and tissue damage. CD105 expression in CRSwNP and EGPA nasal polyps supports the hypothesized involvement of angiogenesis in the pathogenesis and development of nasal polyps.