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Riga-Fede disease (RFD), also known as traumatic eosinophilic granuloma, is a benign inflammatory condition of the oral cavity that may mimic a malignant tumour. It’s a rare condition mainly reported in infants. We present a unique case report of a 57-year-old man who presented to our outpatient department with foreign body sensation in the throat and swelling over the dorsum of the tongue for a period of 1 month. He gave no history of trauma, especially due to sharp teeth. Video laryngoscopy done revealed an ulceroproliferative growth on the midline of the dorsum of the tongue. With clinical suspicion of malignancy, the patient underwent wide local excision of the tongue lesion. Histopathological examination was suggestive of RFD. The postoperative follow-up was uneventful with good wound healing. The patient was followed up with no evidence of recurrence.

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.

BACKGROUND This study aimed to evaluate the long-term clinical and radiologic follow-up results of eosinophilic granulomas (EGs) of the axial and appendicular skeleton managed with biopsy alone. METHODS Fifty-five patients with unifocal osseous EGs of the axial and appendicular skeleton were followed after biopsy. Patients were divided into 2 groups on the basis of localization of the lesions. In group 1, there were 32 (58.2%) children with extremity long bone involvement: femur, tibia, humerus, ulna, and radius. Group 2 included 23 (41.8%) patients with lesions located in other appendicular and axial skeleton bones: pelvis, scapula, clavicle, lumbar, and thoracic vertebrae. After confirming the diagnosis by a closed biopsy, no further surgical intervention was performed. Clinical recovery included regression of the localized symptoms, mainly pain resolution. Functional improvement was assessed by Musculoskeletal Tumor Society (MSTS) scoring. Radiologic healing was defined as ossification of the entire lesion with cortical thickening in long and flat bones, and restoration of vertebral body height in the spine. Complications, including local recurrence, were determined. RESULTS The patients comprised 28 boys and 27 girls with an average age of 9.2 years (range, 3 to 16 y). The average follow-up was 76 months (range, 28 to 132 mo). The median time from biopsy to clinical recovery was 17 days [95% confidence interval (CI), 13.3-20.6] and 36 days (95% Cl, 32.8-39.1) in group 1 and group 2, respectively. MSTS scores increased progressively till the end of 12 months in both groups. The median time from biopsy to radiologic healing was 16 months (95% CI, 11.5-20.4) and 42 months (95% Cl, 39.3-44.6) in group 1 and group 2, respectively. Both clinical recovery (P=0.021) and radiologic healing (P=0.009) were significantly faster in group 1 compared with group 2. No major complication was seen after biopsy. All lesions regressed without a local recurrence. CONCLUSIONS Unifocal osseous EGs have spontaneous healing potential and confirming the diagnosis by biopsy is enough to obtain good clinical and radiologic results without any additional surgical intervention. TYPE OF STUDY This was a therapeutic study. LEVEL OF EVIDENCE Level IV.

We performed gross and histological examinations of the livers of sika deer (Cervus nippon yesoensis) in Hokkaido, Japan. Out of 1,381 deer slaughtered for venison production, thickening and dilation of the large intrahepatic bile ducts and Fasciola flukes in the duct lumens were detected in 621 deer (45.0%). Furthermore, 107 non-bile lesions (75 intrahepatic and 32 capsular lesions) were detected during gross examinations. Histologically, the bile duct lesions included chronic proliferative cholangitis, papillary hyperplasia, goblet cell and pyloric gland metaplasia, and periductal fibrosis. Many of the intrahepatic non-bile duct lesions (53/75, 71%) were considered to be Fasciola fluke migration-associated lesions, including two lesion types: necrosis, hemorrhage, and eosinophilic granuloma formation (29 lesions), and lymphoid tissue formation (24 lesions). Lymphoid tissue formation was considered to result from the persistent immune responses against dead Fasciola flukes. An epidermoid liver cyst was found incidentally, which has not been reported in the veterinary literature. In summary, this study demonstrated the predominance of fascioliasis-associated lesions in sika deer livers. The gross and histological lesions caused by Fasciola flukes in sika deer were similar to fascioliasis in other animals. Moreover, we described lymphoid tissue formation as a fascioliasis-associated lesion for the first time. The fact that bile duct lesions (45.0%) had a markedly higher prevalence than fascioliasis-associated parenchymal lesions (53/1,381, 3.8%) indicated that sika deer are a permissive host for fascioliasis. Our results provide information that will aid pathological examinations of sika deer.

ObjectiveTo develop and validate revised classification criteria for eosinophilic granulomatosis with polyangiitis (EGPA).MethodsPatients 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.ResultsThe 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%).ConclusionThe 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.

To investigate prevalence of anti-Pentraxin 3 (PTX3) antibodies in sera of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) patients.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.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.Anti-PTX3 autoantibodies appear a promising novel biomarker of AAV, especially EGPA.

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.

Author(s): Johnson, Lynelle R; Johnson, Eric G; Hulsebosch, Sean E; Dear, Jonathan D; Vernau, William | Abstract: BackgroundEosinophilic lung disease is a poorly understood inflammatory airway disease that results in substantial morbidity.ObjectiveTo describe clinical findings in dogs with eosinophilic lung disease defined on the basis of radiographic, bronchoscopic, and bronchoalveolar lavage fluid (BAL) analysis. Categories included eosinophilic bronchitis (EB), eosinophilic granuloma (EG), and eosinophilic bronchopneumopathy (EBP).AnimalsSeventy-five client owned dogs.MethodsMedical records were retrospectively reviewed for dogs with idiopathic BAL fluid eosinophilia. Information abstracted included duration and nature of clinical signs, bronchoscopic findings, and laboratory data. Thoracic radiographs were evaluated for the pattern of infiltrate, bronchiectasis, and lymphadenomegaly.ResultsThoracic radiographs were normal or demonstrated a bronchial pattern in 31 dogs assigned a diagnosis of EB. Nine dogs had intraluminal mass lesions and were bronchoscopically diagnosed with EG. The remaining 35 dogs were categorized as having EBP based on radiographic changes, yellow green mucus in the airways, mucosal changes, and airway collapse. Age and duration of cough did not differ among groups. Dogs with EB were less likely to have bronchiectasis or peripheral eosinophilia, had lower total nucleated cell count in BAL fluid, and lower percentage of eosinophils in BAL fluid compared to dogs in the other 2 groups. In contrast to previous reports, prolonged survival (g55 months) was documented in dogs with EG.Conclusions and clinical importanceDogs with eosinophilic lung disease can be categorized based on imaging, bronchoscopic and BAL fluid cytologic findings. Further studies are needed to establish response to treatment in these groups.

Capillaria hepatica (syn. Calodium hepaticum) is a parasitic nematode of rodents, rarely infecting humans. An asymptomatic Israeli adult male with extensive travel history was diagnosed with a liver mass on routine post-thymectomy follow-up. Imaging and computer tomography (CT) guided biopsy were inconclusive. Surgical excision revealed an eosinophilic granuloma with fragments of a nematode suspected to be C. hepatica. Molecular methods verified the diagnosis, and the patient was treated empirically. This is the first case of hepatic capillariasis described in Israel, and the first to be diagnosed using molecular methods.

Background A histiocytosis is a group of immunoproliferative disorders of clonal cells. The management protocols are still evolving, with chemotherapy as the mainstay of treatment. Objective This study aims to evaluate the feasibility, safety, efficacy, and complication profile of stereotactic radiosurgery for intracranial histiocytosis. Methodology The authors reviewed PubMed, Scopus, Web of Science, and Embase for “radiosurgery” and “histiocytosis” in the English/Japanese language following preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines. The patient profile, radiosurgical parameters (dose and isodose), target volume, and mode of radiosurgery (Gamma knife, LINAC radiosurgery, etc.) were collected. Its use as primary or adjuvant therapy, clinical and radiological outcome was also evaluated. Results We identified 7 studies (9 patients); mean age: 41.9 years (24–57 years). Six patients received Gamma-knife radiosurgery, whereas 3 received CyberKnife radiosurgery. The Langerhans cell histiocytosis variants were eosinophilic granuloma in 3, whereas 4 were not defined. Two cases had Rosai-Dorfman disease, and 2 different yet pathogenetically related histiocytic disorders. Four patients harbored lesions in the pituitary stalk and posterior pituitary, 2 patients in the petrous region, 1 patient had a pontine lesion, and 2 patients had multiple lesions. The dose delivered ranged from 8 to 28 Gy. A total of 18 lesions (9 patients) were followed for 81.67 patient-years: 7 (39%) disappeared, 8 (44.4%) showed radiological reduction, and 2 (11%) remained stable. One lesion (5%) showed an increase in size needed surgical excision. There were no adverse effects. Conclusion The role of stereotactic radiosurgery needs to be further evaluated as the current cohort with only 9 cases (2 are Rosai-Dorfman disease) is insufficient to make conclusions. It may be a viable alternative in localized disease, along with chemotherapy and targeted surgery.

Background Vertebra plana in children is a diagnostic dilemma for orthopaedic surgeons. This radiographic finding sometimes has been said to be pathognomonic for eosinophilic granuloma (Langerhans cell histiocytosis); however, vertebra plana may also be caused by a range of other conditions. We sought to determine whether vertebra plana can be associated with malignancies other than eosinophilic granuloma. Questions/purposes (1) To report the underlying diagnoses for children with vertebra plana and determine how frequently these patients were found to have eosinophilic granuloma as opposed to an underlying malignant process, (2) to evaluate the occurrence of nondiagnostic results on biopsy, and (3) to determine whether the presenting characteristics of spinal lesions were associated with the ultimate clinical diagnosis. Methods As part of a retrospective review, our institutional electronic medical record was searched for all patients younger than 18 years between 1976 and 2017 whose clinical record included the term vertebra plana. Patients with trauma were excluded. Twenty-seven patients met the inclusion criteria (mean [range] age 9 years [0 to 18]; 12 girls). To address our first research purpose about the underlying diagnoses of patients with vertebra plana, we reviewed the final clinical diagnosis. To address our second research purpose about the utility of biopsy, we reviewed which patients underwent a biopsy and whether it had been diagnostic. To address our third research purpose about the radiographic criteria, we classified the radiographs and compared this to the clinical diagnosis. Vertebral collapse was described as less than 50% collapse, 50% to 100% collapse, symmetrical, and asymmetrical. The location of each lesion was noted. Results Twelve of 27 patients had a diagnosis of eosinophilic granuloma. Six of 27 had other neoplastic etiologies, including acute lymphoblastic leukemia, primary germ cell tumor, giant cell tumor, rhabdomyosarcoma and teratoma. Seventeen of 27 patients underwent biopsy to confirm the diagnosis; six biopsies were consistent with eosinophilic granuloma, six for other etiologies, and five were nondiagnostic. With the limited patient numbers available, there was no difference in the frequency of less than 50% loss of height or 50% to 100%, or symmetric and asymmetric loss of height, and location of the lesion among patients with eosinophilic granuloma and those with other diagnoses. Conclusions Eosinophilic granuloma or Langerhans cell histiocytosis is a common cause of vertebra plana, but other causes must be considered in children presenting with this radiographic finding. Six of 27 of patients presenting to our center with vertebra plana had an underlying neoplasm other than eosinophilic granuloma. With the limited patient numbers available, pain, spinal location, and the degree and symmetry of collapse were not associated with a diagnosis of eosinophilic granuloma in this series. Thus, patients presenting with vertebral plana and back pain need a comprehensive work-up and potentially tissue biopsy to determine diagnosis and appropriate treatment. Level of evidence Level IV, diagnostic study.

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.

This is a case of a pediatric male who presented with a postauricular mass and pain. Computed tomography and magnetic resonance imaging suggested a diagnosis of Langerhans cell histiocytosis. Characteristic imaging findings and initial workup for Langerhans cell histiocytosis are described.

Eosinophilic granuloma (EG) of spine in pediatric patients presents kinds of clinical manifestation and a difficult management scenario. The choice of treatment, issues of surgical intervention versus conservative treatment, combination therapy or single treatment, all these factors, including neurological deficits, spinal stability, long-term complications, and continued skeletal growth, must be considered. From 2008 to the 2015, 31 pediatric patients of spinal EG were retrospectively reviewed. They were 17 males and 14 females, with a mean age 8.89 ± 2.84 years old (range, 3.5–14 years old). All the cases were divided into two groups. Twenty-three cases (Group S) accepted surgical interventions and surgery combined with local low-dose radiotherapy and/or chemotherapy. Eight patients (Group C) accepted radiotherapy and/or chemotherapy. All the patients had imaging studies of the lesion including standard radiography, three-dimensional computed tomography (CT) scan, and magnetic resonance imaging (MRI) prior to and after treatment. Cases in group S underwent surgery-related treatment, which include six patients accepted surgery only, nine patients accepted postoperative low-dose radiotherapy, four patients accepted additional chemotherapy, and four patients accepted both. Cases in group C accepted radiotherapy and/or chemotherapy, which include five patients accepted radiotherapy, one patient accepted chemotherapy, and two patients accepted both radiotherapy and chemotherapy. Clinical symptoms, neurologic status, radiologic manifestations, treatment, outcome, and/or complications were recorded and analyzed. All the 31 patients had pain relief (both in group S and group C) but the patients in group S obtained more prompt pain relief. All the patients in group S obtained local kyphosis correction, reconstruction of stability of spine, and recovery of neurological deficit after treatment. They have no surgery-related complications after treatment and in follow-up. Seventeen patients who received additional postoperative radiotherapy and/or chemotherapy also had good outcomes. There were no severe radiation and chemotherapy complications in procedure. The patients (group C) who accepted non-surgical treatment also have no severe complications, and a new femur lesion was found in one patient in follow-up. Surgical intervention, including anterior and/or posterior approach with bone graft or surgery combined with postoperative low-dose radiotherapy and/or chemotherapy is a safe and effective way for treatment of the spinal EG. Compared with radiotherapy and/or chemotherapy, more prompt pain relief can be achieved via surgical intervention or surgery combined radiotherapy and/or chemotherapy.

Aim: To investigate the clinical features of five glial fibrillary acidic protein (GFAP) antibody positive patients with suspected benign tumors and explore its underlying pathogenesis. Materials and methods: Overall, 1018 serum and cerebrospinal fluid (CSF) samples were tested by indirect immunofluorescence assay and data from five patients with suspected tumors and positive for GFAP autoantibody in the CSF were analyzed retrospectively. Results: The positive rate of GFAP antibody in the serum and CSF was 3.93% by indirect immunofluorescence assay. Tumors were diagnosed before or after neurologic onset in 5 of 40 patients (12.5%) and no deterioration of the tumors was found during the long-term follow-up. Of the five patients, one patient suffered a thyroid nodule, one patient had a small nodule in the left lung, two patients suffered meningiomas, and one patient had a suspicious eosinophilic granuloma. Conclusion: GFAP autoimmunity may be a paraneoplastic immune response with a low frequency of tumor in Chinese patients with GFAP astrocytopathy.

BACKGROUND: In a recent phase III trial (NCT02020889) 53% of mepolizumab-treated versus 19% of placebo-treated patients with eosinophilic granulomatosis with polyangiitis (EGPA) achieved protocol-defined remission. OBJECTIVE: We sought to investigate post hoc the clinical benefit of mepolizumab in patients with EGPA using a comprehensive definition of benefit encompassing remission, oral glucocorticoid (OGC) dose reduction, and EGPA relapses. METHODS: The randomized, placebo-controlled, double-blind, parallel-group trial recruited patients with relapsing/refractory EGPA receiving stable OGCs (prednisolone/prednisone, ≥7.5-50 mg/d) for 4 or more weeks. Patients received 300 mg of subcutaneous mepolizumab or placebo every 4 weeks for 52 weeks. Clinical benefit was defined post hoc as follows: remission at any time (2 definitions used), 50% or greater OGC dose reduction during weeks 48 to 52, or no EGPA relapses. The 2 remission definitions were Birmingham Vasculitis Activity Score of 0 plus OGC dose of 4 mg/d or less (remission 1/clinical benefit 1) or 7.5 mg/d or less (remission 2/clinical benefit 2). Clinical benefit was assessed in all patients and among subgroups with a baseline blood eosinophil count of less than 150 cells/μL, baseline OGC dosage of greater than 20 mg/d, or weight of greater than 85 kg. RESULTS: With mepolizumab versus placebo, 78% versus 32% of patients experienced clinical benefit 1, and 87% versus 53% of patients experienced clinical benefit 2 (both P < .001). Significantly more patients experienced clinical benefit 1 with mepolizumab versus placebo in the blood eosinophil count less than 150 cells/μL subgroup (72% vs 43%, P = .033) and weight greater than 85 kg subgroup (68% vs 23%, P = .005); in the OGC greater than 20 mg/d subgroup, results were not significant but favored mepolizumab (60% vs 36%, P = .395). CONCLUSION: When a comprehensive definition of clinical benefit was applied to data from a randomized controlled trial, 78% to 87% of patients with EGPA experienced benefit with mepolizumab.

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.

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.

Background The incidence and radiological patterns of eosinophilic granuloma (EG) in China is not clear. We described the incidence, presentation, and imaging characteristics of Chinese EG patients in a tertiary hospital. Methods A retrospective chart review was performed from January 2004 to October 2017 at a single tertiary general hospital. Seventy-six patients were pathologically identified as EG. Besides, 60 patients with preoperative imaging diagnosis of “EG” were analyzed to reveal the radiological patterns and their diagnostic power. Results Fifty-three male and 23 female EG patients with a mean age of 18.1 ± 16.7 years (range 1–58 years) were retrospectively included. Significant differences were observed in gender (male to female = 2.3:1) and age (the highest incidence at the age of 0~5 years) for EG. EG predominantly involved the skeletal system: flat bones (31.43%) > irregular bones (24.76%) > long bones (22.86%) > other organs (20.95%). No obvious relationships between season, biochemical markers, and EG incidence were observed. The common presenting symptoms were pain followed with local mass, and most patients underwent surgical resection. Among 60 imagingly diagnosed “EG” patients from April 2009 to October 2017, only 22 were with histological confirmation. The correct diagnosis rates were 37.1% (13 out of 35), 16.7% (5 out of 30), and 22.2% (8 out of 36) for plain radiography, computed tomography (CT), and magnetic resonance imaging (MRI), respectively. Conclusions Chinese EG has a varied presentation, age distribution, and gender difference. EG diagnosis is still based on biopsy or histopathology instead of imaging techniques. Electronic supplementary material The online version of this article (10.1186/s13018-019-1158-1) contains supplementary material, which is available to authorized users.

Kimura disease (KD) is a rare idiopathic inflammatory disorder of unknown etiology. Unusual presentations of KD might cause diagnostic difficulty or be misdiagnosed as malignancy if clinical suspicion is insufficiently high. Here, we aimed to determine the clinicopathological features of Chinese KD patients to reveal further insights into the natural history and treatment of this disease.The clinical data of 46 cases of KD diagnosed at Peking Union Medical College Hospital from January 1980 to December 2018 were analyzed retrospectively through case record review.Of 46 cases, 40 were male and six were female. The age at onset ranged from 2 to 56 years (median 27 years). All patients presented with either single (26.1%) or multi-focal (73.9%) subcutaneous masses. Twenty-nine (63.0%) cases presented with head and neck subcutaneous masses, and 9 cases (19.6%) involved different parts of the body. Parotid, submandibular, and lacrimal gland involvement occurred in 17 (37.0%), 3 (6.5%), and 2 cases (4.3%), respectively. Nephrotic syndrome was present in three cases (6.5%), and thromboembolism was present in five cases (10.9%). During follow-up, thirteen patients (13/28, 46.4%) relapsed over 1-13 years (median 8.5 years). The recurrence rate in patients receiving corticosteroids, surgery, and combined surgery and radiotherapy was 30.8%, 66.7%, and 50.0%, respectively. One patient was diagnosed with T cell lymphoma 1 year after diagnosis of KD.KD is characterized by subcutaneous masses but it is also a systemic disease. Given the high rate of recurrence and reported association with lymphoma, patients require careful long-term follow-up.Key Points• Kimura disease (KD) is a rare inflammatory disorder of unknown etiology that is endemic in Asia.• Clinicians must regard and manage KD as a systemic disease.• There is no consensus on optimal treatments and further studies are necessary to improve outcomes.• Given the high rate of recurrence and reported association with lymphoma, patients require careful long-term follow-up.

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.

Intracranial Langerhans cell histiocytosis commonly presents as skull lesions in children. An intratumoral cyst with fluid level and epidural hematoma occurring with eosinophilic granuloma is very rare. We report a 15-year-old boy who presented with a spontaneous epidural hematoma which was the result of a temporal eosinophilic granuloma. Multiple explanations for epidural hematoma in such cases have been discussed. Intratumoral hemorrhage followed by cyst formation and rupture may explain the pathophysiology of epidural hemorrhage formation.

// Nan Meng 1 , Min Xia 2 , Ya-Qi Lu 1 , Mi Wang 1 , Krishna M. Boini 2 , Pin-Lan Li 2 and Wang-Xian Tang 1 1 Institute of Liver Diseases, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China 2 Department of Pharmacology and Toxicology, Virginia Commonwealth University, School of Medicine, Richmond, VA, USA Correspondence to: Wang-Xian Tang, email: // Keywords : NLRP3 inflammasome, hepatic stellate cell, cathepsin B, Schistosoma J., Pathology Section Received : February 23, 2016 Accepted : June 01, 2016 Published : June 14, 2016 Abstract The major pathological changes during Schistosoma J. infection are characterized by granulomatous inflammation in the liver, a cellular immune response to schistosomal egg antigens. The molecular mechanisms initiating or promoting this schistosomal granulomatous inflammation remain poorly understood. In the present study, we first demonstrated that in mice infected with Schistosoma J . for 6 weeks exhibited increased levels of IL-1β in liver, a major product of NLRP3 inflammasomes and collagen deposition around the eosinophilic granuloma with Schistosoma J. eggs, which was substantially attenuated by caspase-1 inhibitor, YVAD. This activation of the NLRP3 inflammasome occurred in hepatic stellate cells (HSCs), as shown by a marked increase in co-localization of IL-1β with HSCs marker, desmin. Using isolated, cultured mouse HSCs, we further explored the mechanisms by which soluble egg antigen (SEA) from Schistosoma J. activates NLRP3 inflammasomes. SEA induced the formation and activation of NLRP3 inflammasomes, which was associated with both redox regulation and lysosomal dysfunction, but not with potassium channel activation. These results suggest that NLRP3 inflammasome activation in HSCs may serve as an early mechanism to turn on the inflammatory response and thereby instigate liver fibrosis during Schistosoma J. infection.

Objective To describe the presenting clinical features, treatment and outcome in children with eosinophilic granulomatosis with polyangiitis (EGPA) and to define factors that predicted mortality. Methods A retrospective case notes review of patients fulfilling the Chapel Hill Consensus Conference definition and/or ACR criteria for EGPA seen at Great Ormond Street Hospital, London. Demographics, clinical features, histopathology, treatment and outcomes were recorded. Descriptive statistics were expressed as median and range. Fisher's exact test was used for group comparisons. The Paediatric Vasculitis Activity Score and Paediatric Vasculitis Damage Index (PVDI) were calculated. Results Thirteen children (38% female) aged at diagnosis 14.1 (4-15.6) years were identified. The median time to diagnosis was 2 (0-7.3) years. History of asthma was documented in 76%. The most common presenting features were pulmonary (69%), skin (61%), gastrointestinal (46%), cardiac involvement (46%), paranasal sinus abnormality (38%), arthritis/arthralgia (38%) and neurological involvement (15%). Paediatric Vasculitis Activity Score at presentation was 8/63 (2-25/63); ANCA was negative in all 10/13 patients tested. Treatment included corticosteroids in all, combined with CYC in 38% or AZA in 23%. PVDI at 12 (3-48) months follow-up was 3/72 (0-13/72). Relapses were recorded in 46%. Mortality was 15%; cardiomyopathy and PVDI scores ⩾5 significantly associated with mortality risk (P = 0.012). Conclusion EGPA in the paediatric population is a rare and potentially life-threatening vasculitis. Increased awareness is essential to secure a timely diagnosis and to promptly initiate treatment since our data emphasize a high mortality, particularly in those with cardiac involvement and significant accrued damage.

A large subcutaneous mass, formed on the left lower jaw of a 4-month-old Japanese Black male calf, was partially excised for histological and bacteriological examinations. Antibiotic treatment resulted in a good prognosis. Bacteria isolated from the excised material were characterized by weak hemolysis and positive reactions for catalase and oxidase, and were 99% identical to Mannheimia granulomatis strains. The presence of the leukotoxin gene product was demonstrated by polymerase chain reaction amplification. Histological examination showed that the excised material was composed of dense fibrous connective tissue with sparsely distributed eosinophilic granulomas or abscesses. These foci frequently contained Splendore-Hoeppli material with rod-shaped Gram-negative bacteria. Except for the absence of lymphangitis and the presence of basophils and mast cells, the histology of this lesion resembled that of lechiguana associated with coinfection of M. granulomatis and Dermatobia hominis. Leukotoxin was demonstrated by immunohistochemistry within Splendore-Hoeppli material and was judged to be responsible for its formation.

Background Context Eosinophilic granulomas (EGs) of the sacrum have been reported in fewer than 10 patients. Treatment algorithms for these tumors remain poorly defined; there are no reports of treating solitary sacral EG with radiation therapy (RT). Purpose This study aimed to describe the presentation, treatment, and outcome of sacral EG in an adult patient with intractable pain and radiculopathy, treated in a novel fashion with RT. Study Design/Setting The study design was a case report from a tertiary cancer referral center. Methods Patient records, imaging, and pathology were reviewed. Results A 35-year-old man received 20 Gy of radiation to his S1 EG lesion. He subsequently developed vertebra plana of S1 causing symptomatic L5–S1 stenosis, but 15 months after RT treatment was free of pain or tumor recurrence. Conclusion Radiation therapy is an effective treatment option for sacral EG causing severe axial pain and neural impingement.

Background Benign bone tumors and tumor-like conditions are commonly located in the proximal femur. The main indications for surgical treatment are lesions with impending or actual pathological fractures, or with aggressive or recurrent lesions. However, patients complaining of persistent pain, limping, or abnormal gait patterns are also considered for surgical treatment. In this study, we describe the outcomes of the surgical treatment of benign lytic lesions of the proximal femur by curettage followed by implantation of synthetic bone graft. Methods This retrospective study included 27 patients (22 females and 5 males) with benign lytic lesions of the proximal femur. The average age was 25.5 years (6–65 years), and the mean follow-up period was 54.5 months (9–145 months). The histopathological diagnoses were fibrous dysplasia (8 patients), simple bone cyst (8 patients), chondroblastoma (7 patients), giant cell tumor (3 patients), and eosinophilic granuloma (1 patient). These lesions were managed with curettage followed by implantation of the bone defects with alpha tricalcium phosphate in 14 patients, beta tricalcium phosphate granules in 11 patients, hydroxyapatite granules in 1 patient, and combined beta tricalcium phosphate and hydroxyapatite granules in 1 patient. Internal fixation was performed in three patients. Results The mean operative time was 143 min (80–245 min). Patients had regained normal unrestricted activity without pain at the operation site. Patients treated with beta tricalcium phosphate achieved radiographic consolidation of the bone defects within 1 year after the surgery, and those treated with alpha tricalcium phosphate or hydroxyapatite experienced no progression nor recurrence of the lesions. Local tumor recurrence was observed in one patient with giant cell tumor 5 years after the surgery. Post-operative pathological fracture was occurred in one patient with a simple bone cyst of the subtrochanteric region 1 month after surgery. No post-operative infection was observed. Conclusion We concluded that the treatment of benign lytic lesions of the proximal femur, either primary or recurrent, using synthetic bone graft is a safe and satisfactory method and the addition of internal fixation should be carefully planned.

Background Bone necrosis of the jaw is a serious condition with a broad differential diagnosis of pathologies such as cutaneous histiocytosis, bone metastases or malignant tumours. In addition to the most common cause, medication related osteonecrosis of the jaw (MRONJ), one must consider a number of other causes, such as histiocytosis. Langerhans cell histiocytosis (LCH) is a histiocytic disorder with a large spectrum of clinical manifestations and with possible involvement of a variety of organs. This case shows the importance of an early detection of this rare disease in order to prevent further spreading. Even if an initial diagnosis in the oral cavity is rare, dentists should be aware of this disease. Case presentation The presented case describes a patient who was referred for evaluation and treatment due to exposed bone and extensive osteolysis in the region of the upper and lower jaw. After biopsy and diagnosis of LCH, the patient was treated with systemic therapy, achieved remission and is disease free after a 2 year of follow up. Conclusions This case report illustrates that when dealing with unclear osteolytic changes of the jawbone, Langerhans cell histiocytosis must be taken into consideration in the differential diagnosis and biopsy must be performed in case of suspicion.

Langerhans cell histiocytosis (LCH) is a rare entity formerly known as eosinophilic granuloma. It is characterised by clonal multiplication of langerhans cells (LCs) that can occur anywhere in the body, especially in connective tissue of skin, lymph nodes and bone. Although rare, the disorder frequently affects children. A 7-year male child presented with the complaint of swelling at glabella. CT-scan revealed a defect in the middle of both frontal bones with brain herniating out of it. The clinical differential diagnosis included osteomyelitis, Tuberculosis and encephalocele. Fine needle aspiration cytology (FNAC) was performed and diagnosis of LCH was confirmed by typical cytological features, followed by immuno- histochemical staining for CD1a.

The clivus is a region characterized by complex anatomy, with vascular and neural structures that are located in close proximity. Different pathologies can affect this area, and traditional surgical approaches were open approaches. Recently, the endoscopic transnasal technique has been introduced, and currently represents a good alternative for the surgical management of these lesions. This is a preliminary report on patients treated endoscopically for clival lesions by the authors’ Skull Base Team. This was a retrospective chart review of patients who underwent an endoscopic exclusive transnasal approach (EEA) or a transoral one (TO) for clival lesions between June 2015 and November 2017 at our Skull Base Referral Center. Patient characteristics and symptoms, preoperative neuroradiological evaluation, surgical approach, complications, and postoperative results were evaluated. Nine patients (6 females and 3 males; age range 6–82 years, mean 50.8 years) underwent EEA or TO. From histological analysis, we found chordomas (6/9 subjects), chondrosarcoma (1/9), craniopharyngioma (1/9), and eosinophilic granuloma (1/9). Three patients had previously been operated for a parasellar chondrosarcoma (1/9), a pituitary macroadenoma (1/9), or a chondroid chordoma (1/9). The lesions were totally (2/9) or sub-totally (5/9) resected, debulked (1/9), or analyzed with a biopsy (1/9). Reconstruction was accomplished with a multilayer technique (7/9), or with a gasket–seal (1/9), using a mucoperichondrial graft, a single/double nasoseptal flap, a middle turbinate flap, a fascia lata, or a synthetic fascia. One patient (11.1%) was re-operated on due to cerebrospinal leakage, without further complications. Two patients (22.2%) were re-operated on due to chordoma regrowth. Adjuvant chemotherapy was administered to 1/9 patient with progressive healing. All of the other patients underwent proton-beam radiotherapy with no documented tumor growth (median follow-up: 20 months; range 5.1–29.9 months). Clival lesions represent a heterogeneous group of lesions located in a very complex and difficult area. EEA and TO approaches are safe and mini-invasive, with lower morbidity and with postoperative complications when compared to the traditional open approaches, according to the extent and type of pathology.

Background context Benign tumors of the vertebrae are generally an uncommon cause for surgery. Complete removal of these tumors requires in most cases extensive surgical technique that consists of generous surgical exposure followed by laminectomy, facetectomy, and sometimes even an instrumented fusion. Purpose The aim was to describe our experience in performing resection of benign vertebral tumors, using a minimally invasive surgical (MIS) approach. Study design This was a retrospective review of case records. Patient sample Patients who underwent MIS, resection of benign vertebral tumors. Outcome measures Complete neurologic examination and pain evaluation, as measured by the visual analog scale (VAS). Secondary outcomes included postoperative spinal instability assessment and surgical margins examinations. Methods Patients were evaluated preoperatively and postoperatively at 1, 3, and 6 months intervals clinically and radiographically using plain radiographs and postoperative computed tomography (CT) scans. Final pathologic report, operative time, blood loss, complications, and hospital length of stay were also recorded. Results Between 2009 and 2013, 14 patients underwent MIS, resection of benign vertebral tumors at our institution. Mean follow-up time was 4 years. There were eight men and six women with a mean age of 27 years (range 16–68 years). For tumors located in the posterior elements, a direct posterior approach was used. Tumors located at the pedicle of the vertebra were excised using a transpedicular approach, and tumors protruding into the foramen were excised using the transforaminal approach. The transcanal approach was used when decompression of the thecal sac or nerve root was required, and the retroperitoneal transpsoas approach was used for tumors located in the vertebral body. Complete removal of these tumors was achieved in all cases, and was verified by a follow-up CT scan. Pathology revealed osteoid osteoma in five patients, osteoblastoma in three patients. Eosinophilic granuloma, fibrous dysplasia, and fibroid adenoma were found in one case each. Average VAS pain score improved from 7.7 (7–9) to 2.8 (0–7) after surgery. Conclusions Minimally invasive techniques are a valuable choice for the treatment of benign osseous tumors of the spine. A larger, long-term study is in progress. In the meantime, we suggest surgeons experienced with both open and MIS surgery should consider these techniques.

A 5-year-old intact male, Siberian husky, routinely vaccinated and dewormed for intestinal parasites and ectoparasites was referred with a two-week history of recurrent episodes of respiratory dist...

Delayed swelling after skull fractures is an uncommon complication following head trauma in children. Classically, growing skull fractures typically present in patients under 3 years of age with progressive subcutaneous fluid collections, or occasionally with neurologic symptoms. We present the case of a healthy 2-year-old boy with a lytic "punched-out" frontal skull lesion. The child presented 2 months after a minor forehead injury for which no medical attention was sought.The skull defect had no associated leptomeningeal cyst or brain herniation. Imaging and presentation were thought to be consistent with eosinophilic granuloma. Histologic findings demonstrated a healing skull fracture.Cranioplasty was performed, and the patient had an uncomplicated postoperative course.In this report, we describe our experience with this atypical presentation of a healing skull fracture mimicking a typical eosinophilic granuloma.

Traumatic ulcerative granuloma with stromal eosinophilia (TUGSE) is an underreported diagnosis in dermatologic literature. Rapid expansion with an ulcerative clinical appearance often provokes fear of malignancy despite its benign nature. Traumatic ulcerative granuloma with stromal eosinophilia is thought to be a reactive tissue response to trauma, but CD30

Rationale: Solitary eosinophilic granuloma (EG), the most benign, common form of Langerhans cell histiocytosis, has a self-limiting process and is associated with a good prognosis. Immobilization is recommended as the first treatment strategy for solitary EG, although the treatment protocols are still controversial. Radiotherapy and surgery are secondary treatment choices. Lesions of the upper cervical spine react differently to treatment because of their specific anatomical and motor features. Patient concerns: We discuss the case of a 29-year-old man with axis EG who underwent immobilization, radiotherapy, and finally surgery. Diagnosis: Eosinophilic granuloma (EG). Interventions: An initial conservative protocol, including immobilization with a collar and radiotherapy, effectively relieved his neck pain, whereas torticollis secondary to atlantoaxial subluxation was not improved. Therefore, he underwent tumor resection through the anterior approach and spinal reconstruction, fixation, and fusion through the posterior approach. Outcomes: The deformity was well corrected and follow-up was satisfactory. Lessons: Upon review of the literature, we found that EG lesions affecting the axis have more risk factors for instability or deformity, and they have particular anatomical and motor characteristics; thus, they require more consideration and attention in terms of treatment, prognosis, and follow-up.

Screening for lung cancer with low-dose computed tomography (LDCT) has been shown to reduce mortality and has been recommended by the U.S. Preventive Services Task Force for adults 55 to 80 years of age with a 30 pack-year smoking history who are either current smokers or those that quit within 15 years. However, the overwhelming majority of abnormalities detected are not from malignancy. We report a case of pulmonary Langerhans’ cell histiocytosis, here-to-fore thought of as extremely uncommon, and make readers aware that this may be increasingly found as LDCT is more widely adopted.

Background Nummular headache is a rare, recently described topographic headache defined by the circumscribed coin-shaped area of pain. It is classified as a primary headache. There is debate about whether it is due to a peripheral or central disturbance, and its relationship to migraine. Case reports We report two patients with presumed nummular headache secondary to Langerhans cell histiocytosis, both with resolution of their headaches after surgical resection. Conclusion Imaging in patients with clinical features of nummular headache is recommended, as this and other cases highlight that it may be symptomatic. There are no distinguishing clinical features to separate nummular headache from secondary mimics, and treatment of the underlying cause may be curative.

Introduction Eosinophilic angiocentric fibrosis (EAF) is a slowly progressive, benign disease involving the mucosa of the upper airways or, more rarely, the orbit. It belongs to the spectrum of IgG4-related disease. Case report The authors report the case of a 61-year-old man who presented with orbital involvement (visual loss, pain, proptosis, and eyelid oedema), headache and nasal obstruction. Imaging revealed a right ethmoido-orbital mass infiltrating the periorbital fat and enveloping the optic nerve. Histological examination concluded on a diagnosis of EAF in the presence of perivascular infiltration by inflammatory cells, predominantly eosinophils, and zones of “onion skin” fibrosis. Immunohistochemistry attributed these lesions to IgG4-related disease. Initial treatment with corticosteroids followed by dapsone failed to control the disease and resulted in severe steroid dependence. Surgical ethmoidectomy with resection of the lamina papyracea was performed to allow displacement of the eyeball into the nasal cavity in the event of another episode. Discussion The combination of surgery and rituximab achieved lasting pain relief with no recurrence of exophthalmos.

Background context Eosinophilic granuloma (EG) involving the spine is uncommon in adults. Atlantoaxial instability (AAI) secondary to EG of the axis in adults is an extremely rare clinical condition that can give rise to severe neurologic morbidity or mortality if not treated appropriately. There have been no previous reports on the condition in adults. Purpose To present the outcome and clinical experience for the management of AAI secondary to EG of the axis in adults. Study design A retrospective review study. Patient sample All adult patients with AAI secondary to EG of the axis who were admitted to the spine service at the study institution between January 1999 and April 2012. Outcome measures Clinical symptoms, neurologic status, radiologic manifestations, treatment, outcome, and/or complications were recorded and analyzed. Methods Six consecutive adults who presented clinical and radiographic manifestations of AAI secondary to EG of axis were treated and monitored. All patients were treated surgically with anterior tumor resection and posterior reconstruction of spinal stability. Oral steroid therapy was administered after surgery as adjuvant therapy. Results The mean duration of follow-up was 77 months (range, 37–140 months). The most common radiographic feature was osteolytic destruction of the vertebral body of the axis. All patients had favorable recoveries, with osseous fusion. There were no surgery-related postoperative complications, and neither recurrence nor spinal deformity had occurred by the final follow-up examination. Conclusions Surgical intervention via anterior tumor resection and posterior reconstruction was found to be safe and effective for treating AAI secondary to EG in adults, in terms of recovering neurologic function, improving symptom relief, and reducing the risks resulting from osteolytic destruction. Surgical treatment plus oral steroid therapy can produce beneficial results and definitive local control during the follow-up period.

Salmonellosis is uncommon in cats, usually affects the gastrointestinal tract or skin, and can be fatal. This report describes a domestic shorthair cat with severe pneumonia caused by Salmonella spp. without accompanying gastrointestinal or skin manifestations, in which previous administration of cyclosporine may have played a permissive role in its development. Clinical and laboratory findings as well as follow-up are described from diagnosis until complete recovery. This unusual presentation serves to alert practitioners to consider Salmonella spp. as a possible cause of lung disease in cats, especially if immunocompromised.Salmonellose wird bei der Katze selten erwähnt, befällt häufig Darm oder Haut und kann lebensbedrohlich sein. Diese Arbeit beschreibt eine schwere Pneumonie, verursacht durch Salmonella spp. bei einer Kurzhaar-Hauskatze ohne gastrointestinale oder dermatologische Anzeichen, wobei Cyclosporin als Vorbehandlung ein Risikofaktor bei der Entwicklung der Lungenentzündung gespielt hat. Es werden die klinische Untersuchung, Laborbefunde sowie der Verlauf von der Diagnose bis zur Heilung beschrieben. Dieses seltene klinische Krankheitsbild soll Tierärzte informieren, dass Salmonella spp. als Ursache für eine Lungenerkrankung bei Katzen in Frage kommt, insbesondere wenn sie immunsupprimiert sind.

Mycetoma is a chronic granulomatous infection of skin and subcutaneous tissue, which may involve bone. The disease is caused by true fungi (eumycetoma) or filamentous bacteria (actinomycetoma). Both types of organism are important soil saprophytes, and therefore infection is normally acquired by traumatic inoculation of the skin by contaminated material. Clinically, mycetomas are characterized by tumefaction, draining sinuses and discharging grains. The grains are aggregates of fungal hyphae or bacterial filaments, and grain size, colour and consistency provide the initial clue to the causative organism. To our knowledge, this probably is the first case report of oral eumycetoma of infancy. Review of literature shows only ten cases of eumycetoma involving the head and neck, including the present case. Histological examinations of the biopsies were the cornerstone in the diagnosis of mycetoma. The distinction between eumycetoma and actinomycetoma is essential for treatment. Direct microscopic examination of the grain and histopathological examination, using histochemical staining will distinguish between the two. Treatment consists of long courses of antifungals and antibacterials agents, often combined with surgery.

Information pertaining to parasitic fauna and parasitic diseases in Manipuri ponies in India is not available. Moreover, no systematic studies have been undertaken on cutaneous habronemosis in Manipuri ponies which is a common skin problem of Manipuri ponies as reported by pony owners. Keeping in the view of the importance of parasitic infections in veterinary health coverage particularly in Manipuri ponies, the present study was planned. A survey of natural cases of cutaneous habronemosis followed by molecular confirmation of species involved and treatments were done. Out of 200 ponies examined, nine cases (4.5%) of cutaneous habronemosis was recorded. Gross examination revealed raised and ulcerated wounds with necrotic tissues covered with yellowish-tan granulation. Histopathological study revealed eosinophilic granuloma and in the center of the granuloma with necrotic debris. Remnants of the Hebronema larvae with infiltrating neutrophils surrounded by proliferating fibrous tissue with numerous eosinophils, macrophages and lymphocytes were also observed. Molecular detection of Habronema sp. was confirmed by semi-nested PCR. Sequence analysis revealed larvae of H. muscae was the common spirurid species responsible for producing cutaneous habronemosis in Manipuri ponies. Subsequently, sequence submitted to NCBI GenBank and accession number obtained (MH038181). Surgical removal of necrotic tissue, ivermectin injection along with antibiotics successfully cured all the lesions in infected ponies.Results confirmed occurrence of cutaneous habronemosis in Manipuri ponies in India.

Eosinophilic granuloma (EG) is a rare bony disease deriving from abnormal proliferation of histiocytes, and is the most common form of presentation of Langerhans cell histiocytosis. EG predominantly affects the axial skeleton. However, when localised in the head and neck district, mandibular lesions account for the majority of cases. Mandibular lesions can mimic other pathological conditions, making biopsy fundamental for differential diagnosis. Treatment depends on the severity of the disease, ranging from pharmacological treatment to surgical approach. However, EG is also reported to possibly undergo spontaneous resolution. In this case report, we describe a rare case of EG with particularly aggressive behaviour in a young patient. Initially, local pharmacological treatment with intralesional administration of corticosteroids lead to worsening of the symptoms, increase in lesion’s dimensions, and disruption of the cortical bone. A more invasive therapeutic approach involving radical surgery was then performed, with consequent resolution of the disease.

The granuloma faciale is a rare and benign skin disease of unknown etiology, characterized by chronic leukocitoclastic vasculitis. It is characterized by skin lesions predominantly facial whose course is chronic and slowly progressive. The diagnosis is based on clinical features, histopathology and, more recently, in dermoscopy. We describe the case of a male patient, 40 years old, with a sarcoid lesion on the malar site, whose histopathological examination revealed a mixed inflammatory infiltrate with presence of Grenz zone. Dermoscopy revealed a pink background with white striations. The definitive diagnosis is made by histopathologic evaluation, and dermatoscopy can be helpful. It is known to be resistant to therapy, oral medications, intralesional and surgical procedures are options.

A 43-year-old man reported right visual loss after biopsy of an intranasal mass, which improved after treatment at another hospital. On first examination at our hospital, his visual acuity was 1.2 OD, and MRI showed a right intraorbital mass involving the ethmoid sinus. His visual acuity decreased to 0.08 OD 12 days after orbital biopsy, with right globe tenting shown by MRI. An emergent lateral canthotomy and cantholysis were performed. The histopathological diagnosis was eosinophilic angiocentric fibrosis, and treatment with intravenous methylprednisolone (125 mg/day) and oral diaminophenyl sulfone (75 mg/day) was started immediately after surgery. One month later, the diaminophenyl sulfone was discontinued and cyclophosphamide (50 mg/day) was started. The methylprednisolone and cyclophosphamide doses were tapered over 9 months and 7 months, respectively. At the 1.5-year follow-up examination, his visual acuity was 1.0 OD. The lesions did not grow in size.

Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells intermixed with inflammatory cells, in particular eosinophils, that may manifest as unisystem (unifocal or multifocal) or multisystem disease. Orbital involvement typically manifests as a solitary lesion that carries a favorable prognosis. Herein, we describe the clinical and histologic spectrum of LCH of the orbit on the basis of five cases. One patient exhibited multifocal unisystem disease, the other four patients presented with a localized process. The typical histologic features included numerous histiocytes with varying degrees of giant cell formation and scattered eosinophilic granulocytes. The presence of Langerhans cells was confirmed by CD1a and S100 immunostaining. Transmission electron microscopy exhibited characteristic intracytoplasmic Birbeck granules. The different ophthalmic manifestations of LCH and treatment strategies are reviewed in the context of previously reported cases. As LCH may solely involve the orbit, treatment is based on the degree of organ involvement. LCH has to be included in the differential diagnosis in tumors of the ocular adnexae, in particular in young children.

Study design This was a retrospective clinical study. Objective To evaluate the safety and accuracy of pedicle screw placement in very young children and to observe its influence on vertebral and spinal canal growth. Summary of background data Although widely used, it is not known if pedicle screw fixation is safe and effective in very young children. Methods Sixteen children, with an average age of 34 months, had received pedicle screw fixation from January 2003 to January 2010. Candidates for surgery were those patients who had hemivertebra deformity (11 patients), eosinophilic granuloma disease with spinal cord compression, and neurological deficit (2 patients), or spinal tuberculosis accompanied with kyphotic deformity (3 patients). The location of involved vertebrae was between T2 and L5. A total of 74 pedicle screws were implanted using a modified free-hand technique. The safety and accuracy of this method, and the influence on vertebral growth, was evaluated using postoperative x-ray and computer tomography scans. Result The average follow-up was 30.6 months. No patient had any neurological or radicular symptoms related to the placement of pedicle screws. Postoperative computer tomography scans demonstrated a malposition of 5 of the 74 pedicle screws (6.8%). Two screws breached the anterolateral cortical bone of the vertebral body. One screw breached the lateral cortical bone of the pedicle, 1 passed through the anterior vertebral margin by 3 threads, and a third was so laterally placed that it entered into disk space. There was no vessel, visceral complications, or any other adverse effects resulting from these misplacements. No screw was placed so medially that injured the spinal cord. No retardation of vertebral growth was observed in 7 patients who were followed up for at least 3 and up to 7 years. Conclusions The results indicate that in very young children, pedicle screws can be safely implanted using a modified free-hand implantation technique.

We describe the case of a spontaneous improvement of the cervical kyphosis in eosinophilic granuloma with long-term follow-up. A collapse of the C5 vertebral body was confirmed by cervical spine radiography and computed tomography. The patient wore a sterno-occipitalmandibular immobilizer brace for 6 months, and remodeling of the vertebral body was confirmed 18 months after onset. Seven years have passed since the onset of symptoms, and the patient's cervical spine has maintained normal alignment. Management of eosinophilic granuloma of the pediatric cervical spine is still controversial. Conservative treatment suffices as adequate management of cervical eosinophilic granuloma, even with kyphotic deformity.

Spinal involvement occurs frequently in cases of eosinophilic granuloma (EG), but surgical treatment is limited primarily to those with spinal instability. Involvement of the cervical spine is rare, but primarily occurs in the vertebral bodies, and is normally amenable to anterior corpectomy and spinal reconstruction. The authors describe a 27-year-old man with pathologically proven EG who presented with complete destruction of the C-1 lateral mass requiring spinal stabilization. A titanium expandable cage was used to reconstruct the weight-bearing column from the occipital condyle to the superior articular surface of C-2 from a posterior approach, with preservation of the traversing vertebral artery. To the authors' knowledge, this is the first reported instance of reconstruction of the C-1 lateral mass using an expandable metal cage, which facilitated preservation of the vertebral artery.