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.

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.

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.

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.

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.

Canine eosinophilic pulmonary granulomatosis: case report and literature review.

We describe and illustrate herein a case of eosinophilic pulmonary granulomatosis (EPG) in a 16-mo-old, castrated male, Great Pyrenees crossbred dog. EPG appears to differ from eosinophilic pneumonias and eosinophilic bronchopneumopathy in dogs by the presence of nodules and masses in the lungs composed of eosinophils, macrophages, and various combinations of lymphocytes, plasma cells, neutrophils, and mast cells within fibrous tissue. Specific information from this dog was added to the information from the limited number of other dogs diagnosed with EPG reported in the veterinary literature, and the information analyzed. EPG appears to have no breed or sex predilection and tends to be a disease of younger dogs, often ≤ 3 y of age. Antemortem imaging of nodules and masses in the lungs, combined with cytologic evidence of eosinophils in the lesions, is highly suggestive of EPG, and the diagnosis can be confirmed postmortem. Concurrent eosinophilia and basophilia can be features of EPG, but their diagnostic value requires further investigation, as does the possible causal association with Dirofilaria immitis infection.

Traumatic ulcerative granuloma with stromal eosinophilia - clinical case report, literature review, and differential diagnosis.

BACKGROUND: Traumatic ulcerative granuloma with stromal eosinophilia (TUGSE) is a rare self-limiting condition of the oral mucosa. The lesion manifests as an isolated ulcer that can be either asymptomatic or associated with mild to severe pain, and in most cases, it affects the tongue. TUGSE lesions may mimic malignancy such as squamous cell carcinoma, CD30 positive lymphoproliferative disorder, or infectious diseases such as primary syphilis, tuberculosis, or Epstein-Barr virus mucocutaneous ulcer. Histologically dominating cells are lymphocytes, histiocytes, and eosinophils. CASE PRESENTATION: We describe a TUGSE case of a patient with a solitary ulcer on the lower left retromolar buccal plane. Upon presentation, the patient reported a swelling on the buccal mucosa of the left lower jaw since 1 year with rapid growth over the last days and mild pain while chewing. The diameter of the intraoral lesion on the lower left retromolar buccal plane was approximately 4 × 3 cm; the lesion presented as indurated base with a central superficial ulceration of 2 × 1 cm, indicative for a malignant process. Histologically, the ulceration showed an expanding, infiltrative, and vaguely granulomatous morphology, involving the superficial mucosa and the fatty tissue, and extended between the deep striated muscle fibers. The lesion was rich in lymphocytes, histiocytes, and eosionophils intermingled with activated T-blasts without phenotypic abnormalities. TUGSE was then diagnosed based on the phenotype (especially the lacking expression of CD30, the retained T-cell phenotype, and the absence of Epstein-Barr virus), the clinical presentation, and the morphology. Twenty-six months after diagnosis, no recurrence of the ulceration was seen. CONCLUSIONS: As TUGSE may mimic malignancy or infectious diseases, biopsy is mandatory and should be combined with thorough clinical examination. A screening for infectious diseases (mainly syphilis, Epstein-Barr virus, and HIV infections) must be performed routinely. In most cases, the lesions resolve spontaneously, obviating the need of further actions other than clinical follow-up. The pathogenesis of TUGSE lesions is still under debate, although local traumatic events and a locotypic immune response have been suggested to be major contributing factors.

Angiomatoid Fibrous Histiocytoma Mimicking Eosinophilic Granuloma in a Pediatric Patient.

BACKGROUND: Angiomatoid fibrous histiocytoma (AFH) is a rare low-grade malignant tumor mainly occurring in soft tissues, and its incidence in the bones is extremely rare. Although most of the existing reports focus on the pathological features of AFH, only a few describe its imaging features. To our knowledge, this is the first case of AFH in the skull, and it is distinguished from eosinophilic granuloma based on imaging results. CASE DESCRIPTION: A boy aged 10 years presented with a painless mass of parietal bone after trauma. Cranial computed tomography angiography showed local bone defects near the sagittal suture of the left parietal bone and a soft tissue mass with relatively uniform density in the same area. The signals of this mass were heterogeneous in all sequences of magnetic resonance imaging and presented septal enhancement after the injection of contrast agent. The patient underwent complete resection of the mass, and the histopathological and immunohistochemical diagnosis of the mass was AFH. No complications occurred after the operation and no recurrence occurred during the follow-up. CONCLUSIONS: To our knowledge, this is the first AFH that occured in the skull, and the main imaging manifestations of AFH are bone destruction with soft tissue mass. The characteristic features of AFH are its fibrillar component that showed low signal on T2-weighted imaging and septal or peripheral enhancement, and no dead bone in the mass.

Clinicopathological Features, Risk Factors and Predispositions, and Response to Treatment of Eosinophilic Oral Disease in 24 Dogs (2000-2016).

The objectives of this study were to retrospectively describe clinicopathological features of eosinophilic oral disease in dogs, to identify possible risk factors or predispositions to the condition, and to report overall treatment response. Canine medical records from a veterinary teaching hospital and private referral practice over a 17-year period were reviewed for a diagnosis of eosinophilic oral disease. Twenty-four dogs with 26 lesions met the inclusion criteria. Patient mean age and body weight were 6.8 (3.8) years and 13.4 kg, respectively. Fifteen breeds were represented including Cavalier King Charles spaniel (16.7%), Labrador retriever (12.5%), and West Highland white terrier (12.5%). Eosinophilic lesions were found in the palate (65.4%), tongue (26.9%), and other oral locations (7.7%). Median follow-up time was 5 months. Analysis revealed statistically significant associations between lesion location and body weight (palatal and tongue lesions were more likely in smaller dogs, whereas lesions in the other category [lip or mucosa] were more likely in larger dogs). There was a correlation in lesion location and resolution (all dogs with palatal lesions became asymptomatic at their last recheck), and resolution and the use of antibiotics plus prednisone (greater likelihood of resolution without the use of this combination). Seventy percent of asymptomatic dogs resolved without medication or with allergen therapy alone, suggesting that asymptomatic dogs may respond well to conservative management. No associations were found between lesion location and breed, signalment and response to therapy, lesion resolution and the use of glucocorticoids, or significance of peripheral eosinophilia.

Langerhans cell histiocytosis presenting as eosinophilic granuloma of the bilateral forearms in an 8-year-old girl: a case report.

BACKGROUND: Langerhans cell histiocytosis previously known as histiocytosis X is a rare disease of children and young adults with a very broad clinical spectrum. In children, its annual incidence is estimated between 0.2-0.5 per 100,000. CASE REPRESENTATION: An 8-year-old Moroccan girl with no known personal or family history presented to our institution with painful swelling of both forearms. An X-ray and magnetic resonance imaging were inconclusive. We then performed a biopsy curettage (of her left forearm). Microscopic analysis followed by immunohistochemical analysis disclosed a diagnosis of Langerhans cell histiocytosis. No chemotherapy was necessary. Clinical and radiological improvement was achieved after 6 months. CONCLUSION: The particularity of this observation is the bilaterality of the lesion on both forearms and it has not previously been reported. Langerhans cell histiocytosis should be included in the differential diagnosis of osteomyelitis and Ewing's sarcoma.

Multidisciplinary rehabilitation of eosinophilic granuloma with bone graft surgery and a modified implant-supported hybrid prosthesis: A case report with a 6-year follow-up.

Eosinophilic granuloma is the most common form of Langerhans cell histiocytosis and corresponds with bone lesions characterized by pain, rapid growth, and high tendency of recurrence after inadequate curettage. It is a rare disease that is difficult to diagnose clinically and radiographically because it mimics other odontogenic cysts and tumors. In this report, the reconstruction of an osseous defect with an iliac graft and a modified implant-supported hybrid prosthesis after surgical excision of an eosinophilic granuloma in the mandible of a 27-year-old male patient was described. The patient was satisfied with the functional and esthetic results of the implant-supported restoration and a 6-year follow-up showed no sign of recurrence.

Serum periostin as a biomarker in eosinophilic granulomatosis with polyangiitis.

OBJECTIVE: Identification of a biomarker for disease activity in eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss) remains an unmet need. This study examined the value of serum periostin, a marker of type 2 inflammation, as a measure of disease activity in patients with EGPA. METHODS: Participants enrolled in a multicenter, prospective cohort of patients with EGPA were included in this study if they had disease activity (defined as Birmingham Vasculitis Activity Score [BVAS] > 0) during follow-up. Serum levels of periostin were measured at flare visit as well as two pre- and two post-flare visits, if available. The outcome of disease activity was assessed either with BVAS or Physician Global Assessment (PGA). Mixed-effect models were used to examine the association between periostin levels and disease activity. Comparisons were made with a historical cohort of healthy individuals and patients with asthma. RESULTS: In the 49 patients included in the study, the median periostin level was 60 ng/ml (IQR 50 to 73) in all visits and did not significantly change across visits. Multivariate analyses found no association between periostin level and presence or absence of flare according to the BVAS (adjusted OR 1.00 [95% CI 0.98 to 1.02], p = 0.98) but an increase in periostin level was significantly associated with greater disease severity during a flare according to the PGA (adjusted beta-coefficient 0.02 [95% CI 0.004 to 0.03], p = 0.01). Periostin levels in EGPA were significantly higher than previously studied healthy controls and patients with asthma. CONCLUSION: In EGPA serum periostin level is modestly associated with greater disease severity during a flare but does not discriminate active from inactive disease. Periostin levels in EGPA are higher than in other previously studied cohorts, including healthy populations and patients with asthma, and are relatively stable over time.

Mixed histiocytosis: A case report and published work review.

Histiocytoses are a group of heterogeneous diseases that encompass Langerhans cell histiocytosis and non-Langerhans cell histiocytosis. Cutaneous plane xanthoma is a non-Langerhans cell histiocytic disorder characterized by the presence of yellow-orange plaques on the face, neck, upper trunk and extremities. It can appear in association with several systemic diseases (including dyslipidemias, paraproteinemias, cardiovascular diseases and lymphoproliferative disorders), but is rarely connected with Langerhans cell histiocytoses. Eosinophilic granuloma is one of the clinical entities of Langerhans cell histiocytoses, characterized by skeletal lesions and occurring prominently in children. Mixed histiocytosis, the concomitant occurrence of Langerhans cell histiocytosis and non-Langerhans cell histiocytosis in a single patient, is exceptional. We herein report a case of eosinophilic granuloma in an adult Chinese man who also developed plane xanthoma on his scalp and face, and we also include a published work review of the comorbid cases of eosinophilic granuloma and non-Langerhans cell histiocytosis. To the best of our knowledge, this is the first report on the mixed histiocytosis of cutaneous plane xanthoma and eosinophilic granuloma in China.

Abdominal Angiostrongyliasis: A Presentation of Eosinophilic Granulomatous Colitis.

We present a case of a 4-year-old girl with abdominal angiostrongyliasis who presented with persistent fevers, hepatosplenomegaly, acute abdominal pain, and eosinophilia. Computed tomography scan identified thickening of the ascending colon with a narrowed lumen. Endoscopic evaluation revealed ulcerations and erythema in the ascending colon. The microscopic findings in biopsies included active chronic inflammation with prominent eosinophils and granulomas. A subset of granulomas contained the eggs of Angiostrongylus costaricensis. The definitive method of diagnosing A costaricensis is histology; peripheral blood serology has low specificity and the stool from infected patients does not contain eggs or larvae. Pathologists from endemic regions (Central and South America) are familiar with the typical histologic changes; however, because of increasing global travel, all pathologists should become familiar with A costaricensis, which may mimic common gastrointestinal diseases such as Crohn's disease, appendicitis, and Meckel's diverticulum.

Eosinophilic Granuloma of Mandible: A Diagnostic Challenge.

Eosinophilic Granuloma is the mildest and localized form of Langerhans Cell Histiocytosis and is characterized by clonal proliferation of Langerhans cells. It is a rare disease, accounting for less than 1% of all the osseous neoplasms. It has predilection for the axial skeleton and incidence in jaws is just 7.9%. It lacks pathognomonic clinical and radiographic trait and hence is difficult to make a correct diagnosis without histopathological and immunohistochemical examination. This report describes a case of Eosinophilic Granuloma of mandible in 30 years old male who presented with complain of unhealed extraction wound and was clinically diagnosed as chronic suppurative osteomyelitis. The final diagnosis of Eosinophilic Granuloma was made only after histopathological and immunohistochemical evaluations.