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A 60-year-old woman was admitted to the hospital with worsening dyspnoea, cough and chest pain. This was on a background of weight loss, decreased appetite, mononeuritis multiplex, chronic eosinophilia and a single episode of a non-blanching rash. Investigations demonstrated a raised troponin and ischaemic changes on ECG, and she was therefore initially treated for a presumed myocardial infarction. However, her symptoms failed to improve with treatment for the acute coronary syndrome. A coronary angiogram revealed no significant flow-limiting disease, and further investigations yielded confirmation of raised eosinophils and a positive perinuclear antineutrophil cytoplasmic antibody test. An echocardiogram demonstrated a pericardial effusion, and subsequent cardiac magnetic resonance features were compatible with myopericarditis. In light of these findings, the patient was diagnosed with eosinophilic granulomatous with polyangiitis and commenced on high-dose intravenous methylprednisolone and cyclophosphamide. She made an excellent recovery and remains in remission on azathioprine and a tapering dose of corticosteroids., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

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., (Copyright © 2018 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

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: Canine eosinophilic granuloma (CEG) is an uncommon disease. Lesions are typically located in the oral cavity and other cutaneous sites, but are rarely reported to affect the digits. The majority of cases are treated with prednisolone as a monotherapy; alternative treatment options include corticosteroids administered in combination with azathioprine, antihistamines, electrochemotherapy with bleomycin, and surgical resection. Neither chlorambucil nor laser previously have been reported as treatments., Objectives: To describe an alternative therapy for treatment of CEG; using chlorambucil in combination with prednisolone for those cases that fail to respond to prednisolone alone. The new treatment was chosen according to good clinical practice and after owner consent., Animals: Two client owned dogs., Methods: One case was initially treated with carbon dioxide laser to debulk the lesions. Both cases were treated with a combination of oral prednisolone and chlorambucil., Results: Both dogs experienced rapid resolution of lesions with prednisolone and chlorambucil therapy. Case 1 remained in remission three months after withdrawing medication. Case 2 experienced relapse 10 weeks after discontinuing therapy but was well controlled on maintenance prednisolone with chlorambucil at low, well tolerated doses., Conclusions and Clinical Importance: Although CEG appears to be an uncommon disease, it should be included as a differential diagnosis for dermal, nodular lesions affecting the digits. Chlorambucil appears to be an effective and well tolerated prednisolone sparing agent for treatment of CEG. Carbon dioxide laser ablation appears to be an effective method of debulking CEGs., (© 2016 ESVD and ACVD.)

Considering the immune system dysfunction in unifocal orbital eosinophilic granuloma; possible complications of surgery, intralesional steroid, and radiotherapy; immunomodulatory and cytoreductive effects of interferon-α2b; and its safety profile in treatment of ocular surface neoplasia, intralesional interferon-α2b was first commenced in treatment of 3 cases (8-, 25-, and 43-year-old men) with biopsy proven orbital eosinophilic granuloma (2007-2014). Three intralesional injections of interferon-α2b (1.5 million units/0.5 ml) were given every other day. Cases 1 and 3 showed a rapid response with no recurrence in 86 and 29 months follow up. Case 2, however, required a second course of injection 2 months after the first one due to a partial response to the first injections with no recurrence at last follow up (57 months). Transient flulike symptom was the only side effect that was observed in Cases 2 and 3.

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., (© The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Purpose: To report 2 cases of Splendore-Hoeppli phenomenon, a rare histopathologic observation, as a late clinical finding in vernal keratoconjunctivitis (VKC)., Methods: We report the cases of 2 young women with subconjunctival nodules as a manifestation of Splendore-Hoeppli phenomenon with a history of severe VKC., Results: After eliminating other conditions causing such a reaction, both patients were treated using frequent topical corticosteroid, plus topical cyclosporine 2% in one of patient. Complete resolution was observed in both patients., Conclusions: The Splendore-Hoeppli phenomenon could be a part of VKC manifestations.

Granuloma faciale is a rare dermatosis of chronic course, benign, usually asymptomatic, first described in 1945 by Wingley. It is characterized by the appearance of well-defined, single or multiple papules, plaques and nodules, predominantly located in sun-exposed areas, especially the face. In this work we report the case of a female patient, 58 years old, evolving for ten years with multiple erythematous-brownish and asymptomatic papules on the face, whose histological examination confirmed the diagnosis of granuloma faciale. The patient was treated with topical tacrolimus, evolving with regression of lesions.

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.

A 13-year-old girl presented headache for 5 d upon admission to hospital. An initial CT revealed 3 lesions located in her skull, the sizes of which were 2.5 cm×3.2 cm,1.2 cm×1.0 cm,0.3 cm×0.3 cm, respectively. The largest lesion was resected by surgery and confirmed as eosinophilic granuloma by pathology. After surgery, she took oral indomethacin 25 mg b·i·d for 3 months and tolerated it well. CT scan was performed 3 months and 1 year later, and the results showed that the unresected lesions shrank progressively and the defected bones were regenerated and healed one year later after operation.

Object: Atlantoaxial tumors account for a substantial proportion of primary bone tumors in children. Before resection, surgeons must consider the complex regional anatomy, the potential for neurological compromise, craniocervical instability, and the question of tumor resectability in a growing spine. Using current technology, the authors analyzed surgical cases in this light and present outcomes and treatment recommendations after long-term patient follow-up., Methods: The authors reviewed clinical records for 23 children whose primary atlantoaxial bone tumors were treated from 1996 through 2010., Results: Pathological lesions among the 23 patients were 4 aneurysmal bone cysts, 2 osteochondromas, 5 chordomas, 4 osteoblastomas, 3 fibrous dysplasias, 4 eosinophilic granulomas, and 1 Ewing's sarcoma. Clinical presentation consisted of neck pain (n = 23), headaches and occipital pain (n = 16), myelopathy (n = 8), and torticollis (n = 4). Selective angiography and coil embolization were undertaken for all patients with aneurysmal bone cysts and osteoblastomas, 2 patients with chordomas, 1 patient with fibrous dysplasia, and 1 patient with Ewing's sarcoma. Primary embolization treatment of radiation-induced aneurysmal bone cyst of the atlas showed complete reossification. Results of CT-guided needle biopsy were diagnostic for 1 patient with eosinophilic granuloma and 1 with Ewing's sarcoma. Needle biopsies performed before referral were associated with extreme blood loss for 1 patient and misdiagnosis for 2 patients. Surgery involved lateral extrapharyngeal, transoral, posterior, and posterolateral approaches with vertebral artery rerouting. Complete resection was possible for 9 patients (2 with osteochondroma, 3 with fibrous dysplasia, 2 with chordoma, and 2 with osteoblastoma). Decompression and internal fusion were performed for 3 patients with aneurysmal bone cysts. Of the 23 patients, 7 underwent dorsal fusion and 4 underwent ventral fusion of the axis body. Chemotherapy was necessary for the patients with eosinophilic granuloma with multifocal disease and for the patient with Ewing's sarcoma. There was no morbidity, and there were no deaths. All patients with benign lesions were free of disease at the time of the follow-up visit (mean ± SD follow-up 8.8 ± 1.1 years; range 2-18 years). Chordomas received proton or LINAC irradiation, and as of 4-15 years of follow-up, no recurrence has been noted., Conclusions: Because most atlantoaxial tumors in children are benign, an intralesional procedure could suffice. Vascular control and staged resection are critical. Ventral transoral fusion or lateral extrapharyngeal fusion has been successful. Resection with ventral fusion and reconstruction are essential for vertebral body collapse. Management of eosinophilic granulomas must be individualized and might require diagnosis through needle biopsy.

The Splendore-Hoeppli reaction is a rare phenomenon characterised by the formation of eosinophilic material around infectious or non-infectious agents. A 33-year-old patient with persistent Splendore-Hoeppli phenomenon was previously treated with topical steroids but this resulted in a rise in intraocular pressure. The patient was treated with topical cyclosporin A one per cent twice daily as an alternative immunosuppression. After three weeks of treatment the patient had complete resolution of her conjunctival granuloma. This case report introduces cyclosporin A as a treatment option for the Splendore-Hoeppli phenomenon. This is a safe drug for long-term topical use in this condition., (© 2013 The Authors. Clinical and Experimental Optometry © 2013 Optometrists Association Australia.)

Langerhans cell histiocytosis (LCH) is a group of relatively rare disease processes of reticuloendothelial system with an abnormal proliferation of Langerhans cells or their precursors. A wide spectrum of treatment modalities is available for LCH which includes surgery, curettage, steroids, radiation, various chemotherapy regimens, either in combination or alone have so far been adopted. There are several case reports of eosinophilic granuloma but for recurrent cases very few have discussed on treatment of LCH in adults. We report a case of a 21-year-old man presented with eosinophilic granuloma with recurrence of the lesions after 6 years of the initial treatment. Patient was treated with combination chemotherapy with three weekly CHOP regimen and the patient is disease free after 6 months of follow-up. Contrary to other reports, this case demonstrates that a good response with standard therapy is possible.

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.

Our aim is to provide review of available studies on Langerhans cell histiocytosis (LCH) and discuss treatment for polyostotic monosystem form of disease based on our clinical experience. LCH is an enigmatic disease with insufficiently understood etiology, pathophysiology, and variety of clinical presentations ranging from solitary eosinophilic granuloma to severe multisystem disease. It is marked by formation of granuloma in practically any organ. We present rare case of multifocal bone disease in 36-year old patient without visceral involvement. Treatment protocols for adult LCH patients, especially for uncommon form in our case have not yet been defined. Our therapeutical trial with corticosteroids showed limited success with numerous side-effects. We conclude that LCH treatment can commence only after diagnosis and staging of the disease. Other factors like patient's age, comorbidity, general condition, severity of symptoms and contraindications for therapy modalities should also be considered. In our experience expectative approach has better clinical outcome than immunosuppressive therapy in patients suffering from polyostotic multifocal form LCH with mild symptoms.

A two-year-old Jack Russell terrier was presented for evaluation of chronic cough and exercise intolerance. Previous treatment with antibiotics and glucocorticoids had only partially ameliorated the clinical signs. During investigation, hypoxaemia, peripheral eosinophilia and an eosinophilic bronchoalveolar lavage fluid were noted. Thoracic radiographs revealed two ovoid clearly delineated soft-tissue opacities, one in the caudal segment of the left cranial lung lobe (diameter 26 mm) and the other in the right cranial lung lobe (diameter 20 mm). These findings were verified by computed tomography, which identified an additional smaller lesion (diameter 16 mm) dorsally in the right caudal lobe. Ultrasound-guided fine-needle aspiration samples confirmed the diagnosis of eosinophilic pulmonary granulomatosis and treatment with prednisolone and azathioprine was initiated. Within 1 month, granulomas were no longer detectable radiographically. All medication was discontinued after 7 months and currently, after 2·5 years, the dog remains free of clinical signs. To the authors' knowledge this is the first case report to describe prolonged remission from idiopathic canine eosinophilic pulmonary granulomatosis., (© 2012 British Small Animal Veterinary Association.)

Two women, 66-year-old and 63-year-old, were admitted for drastically progressive neuropathy, and diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA). Steroid pulse therapy failed to exert effect upon neuropathy, so we administered early intravenous immunoglobulin (IVIG) for fear of immobilization of neuropathy. This resulted in marked improvement in neuropathy without apparent side effects. Recent evidence suggests the efficacy of IVIG for steroid refractory neuropathy associated with EGPA, but has previously been administered during the chronic phase resulting in slow improvement. Our two successfully treated cases indicate the efficacy of early IVIG in preventing the immobilization of neuropathy, especially in progressive cases.

Background: Previous case reports and small series have reported on the treatment of eosinophilic granuloma of bone. We present our long experience in a large group of children and teenagers with symptomatic eosinophilic granuloma of the appendicular skeleton to evaluate clinical and imaging outcome after methylprednisolone injection., Methods: Sixty-six patients with symptomatic solitary eosinophilic granuloma of the appendicular skeleton treated by incisional or percutaneous biopsy and methylprednisolone injection were retrospectively studied. There were 38 boys and 28 girls (mean age, 7.2 y). The mean follow-up was 10.7 years (median, 11.2 y; range, 3 to 15 y). All patients presented with symptomatic lesions including pain or tenderness and fever and had 1 intralesional injection of methylprednisolone acetate after biopsy: 52 patients had incisional biopsy and 14 patients had percutaneous computed tomography-guided biopsy., Results: Complete resolution of symptoms was observed in 58 patients (92%) at 48 to 72 hours (50 patients) and in 7 days (8 patients) after the procedure. Complete imaging reconstitution of bone was observed in 60 patients (95.2%) at 1 to 2 years after the procedure. No patient had recurrence. Multifocal disease was diagnosed in 7 patients (11%) at 3 months to 6 years. Complications occurred in 2 patients: one patient with a clavicular lesion had a pathologic fracture after open direct methylprednisolone injection and the second patient developed trochanteric bursitis after computed tomography-guided methylprednisolone injection., Conclusions: Biopsy and direct intralesional methylprednisolone injection is safe for symptomatic eosinophilic granulomas of the appendicular skeleton in children with effective clinical and imaging resolution of the lesions.

Introduction: Eosinophilic granuloma (EG) is a benign bone tumor that rarely occurs in adults. It is usually found occurring in flat and long bones, but spine is often affected too. EG is of unknown aetiology, and the course of the disease is unpredictable., Materials and Methods: Two cases of EG of the adult lumbar spine are reported, representing a difficult challenge for diagnosis and treatment., Conclusion: CT scan guided trocar biopsy allowed to achieve a definitive diagnosis, and CT scan guided intralesional infiltrations of corticosteroids were the successful treatment.

Objective: We studied the effects of intravenous immunoglobulin (IVIG) treatment on clinical symptoms and regulatory T (Treg) cell frequency in patients with eosinophilic granulomatosis with polyangiitis (EGPA)., Methods: Twenty-two EGPA patients with severe mononeuritis multiplex or cardiac dysfunction received IVIG therapy combined with conventional therapy (corticosteroid, immunosuppressants, or both). As a control, 24 EGPA patients without severe vasculitic symptoms were treated with conventional therapy. Before, during, and after treatment, we determined percentages of Treg cells and other relevant cells in patients' peripheral blood., Results: The frequency of CD25+ among CD4+ T cells was lower at onset in the study group than in controls but increased significantly after IVIG treatment, relative to controls. The frequency of CD25+ among CD4+ T cells correlated with the frequency of FOXP3+ among CD4+ T cells and interleukin 10 produced by CD25+CD4+ T cells., Conclusion: The increase in Treg cells seen with the combination of IVIG and conventional therapy may promote remission in EGPA.

In this study, we evaluated the treatment of feline eosinophilic plaques and lip ulcers with amoxicillin trihydrate-potassium clavulanate (Clavamox(®); Pfizer Animal Health). Nineteen cats with clinical and cytological findings consistent with eosinophilic plaques and/or lip ulcers were enrolled. Lesions were photographed and their areas measured in square centimetres before and after 21 days of therapy with either flavoured amoxicillin-clavulanate suspension or flavoured placebo suspension. Sixteen cats completed the study, with nine plaque lesions (four treatment and five placebo) and eight lip ulcer lesions (four treatment and four placebo) included in the analysis. All lesions were shown to have infection, with bacterial phagocytosis present on cytological examination. Coagulase-positive staphylococci were the most commonly isolated bacteria. The amoxicillin-clavulanate-treated eosinophilic plaque group had a statistically significant 96.2% reduction in mean lesion size (-7.60 cm(2), P = 0.0078) and an 80% reduction in mean percentage of microscopic fields demonstrating evidence of bacterial infection (P < 0.0001), whereas the placebo group did not. The amoxicillin-clavulanate-treated lip ulcer group had a 42.6% decrease in mean lesion size (-0.25 cm(2), P = 0.4125) and the placebo group a 36.6% increase (+0.49 cm(2), P = 0.1575), although neither change was statistically significant. The amoxicillin-clavulanate-treated lip ulcer group had a statistically significant 65.0% reduction in mean percentage of microscopic fields demonstrating evidence of bacterial infection (P < 0.0001), while no significant reduction was observed in the placebo group. A suspension of amoxicillin trihydrate-potassium clavulanate is an effective monotherapy for the treatment of feline eosinophilic plaques., (© 2011 The Authors. Veterinary Dermatology. © 2011 ESVD and ACVD.)

Objective: To evaluate the clinical and imaging outcome of patients with symptomatic eosinophilic granuloma of the spine treated with CT-guided intralesional methylprednisolone injection after biopsy., Materials and Methods: Patients (n =19) with symptomatic solitary eosinophilic granuloma of the spine treated by CT-guided intralesional methylprednisolone injection were retrospectively studied. There were 12 males and seven females with a mean age of 17 years (range, 3-43 years). The mean follow-up was 6 years (median, 4 years; range, 0.5-19 years). Spinal location included the cervical (two patients), thoracic (seven patients), lumbar spine (eight patients), and the sacrum (two patients). Vertebra plana was observed in two patients. All patients had biopsies before treatment., Results: Complete resolution of pain and healing of the lesion was observed in 17 patients (89.5%); none of these patients had recurrence at the latest examination. Reconstitution of the T1 and L1 vertebra plana was observed in both patients. Two patients initially diagnosed and treated for a solitary eosinophilic granuloma had constant pain after the procedure; in these patients, 6 and 12 months after the procedure, respectively, imaging showed multifocal disease and systemic therapy was administered. Complications related to the procedure were not observed. General anesthesia was administered in two patients because of intolerable pain during the procedure., Conclusions: In view of the benign clinical course of eosinophilic granuloma, in patients with symptomatic lesions, CT-guided intralesional corticosteroid injection is a safe and effective outpatient treatment with a low complication rate.

Observation and immobilization is adequate for most patients with spinal eosinophilic granuloma; however, in patients with symptomatic lesions, treatment other than simple observation or biopsy alone is recommended. In view of the benign clinical course of eosinophilic granuloma, a simple, minimally invasive, outpatient treatment with a low complication rate such as computed tomography (CT)-guided intralesional corticosteroid injection may be considered the treatment of choice. This article presents a case of a 21-year-old man with a symptomatic bifocal eosinophilic granuloma at the vertebral body of the L3 vertebra and the left T5 costovertebral joint treated effectively by CT-guided intralesional methylprednisolone injection. To the best of our knowledge, this is the first case of successful treatment of a bifocal eosinophilic granuloma with CT-guided corticosteroid injection. Under general anesthesia and CT guidance, the lesion was located through the right transpedicular approach for the L3 lesion and the left posterolateral approach for the T5 lesion using a biopsy trocar. Tissue sample was retrieved and frozen section biopsy showed eosinophilic granuloma. One intralesional CT-guided injection of 80 mg (2 mL) of methylprednisolone acetate was performed in each lesion. Complications related to the procedure were not observed. The patient was admitted postprocedural for overnight medical evaluation and was discharged from the hospital the next day. Complete resolution of pain was observed 72 hours after the procedure. At 5 years after diagnosis and treatment, the patient is asymptomatic; imaging showed healing of both lesions., (Copyright 2011, SLACK Incorporated.)

Eosinophilic ulcer of the oral mucosa is a benign lesion of unclear pathogenesis mostly affecting the tongue. It has been suggested to represent a reactive pattern to several stimuli. We report on a 12-year-old boy who presented with a painless infiltrating ulcer on the gingiva of the lower jaw, which was covered by necrotic yellowish slough. There were no pathologic features of the jawbones or regional lymph nodes. Histopathological, immunohistochemical and gene rearrangement studies were in agreement with eosinophilic ulcer with predominant oligoclonal CD3+ and CD30+ T lymphocytes expressing the Epstein-Barr virus membrane protein. The ulcer resolved within 4 weeks and follow-up for 3 years revealed no evidence of recurrence. Epstein-Barr virus may have played a role in triggering this reactive lymphoproliferative disorder., (Copyright © 2011 S. Karger AG, Basel.)

Localized Langerhans cell histiocytosis, also known as eosinophilic granuloma (EG), is a benign tumour-like inflammatory disease with variable clinical course. The peak incidence of EG is between five and ten years of age. EG in children down to the age of two years has been described. To our knowledge congenital EG has not previously been described. We present a case of congenital EG.

Background: We compared indomethacin therapy with the more aggressive approaches of anti-cancer chemotherapy and surgery in the treatment of isolated Langerhans cell histiocytosis (LCH) of bone in children., Methods: Comparisons were made with respect to healing of the lesion without recurrence, time to radiological healing of the lesion, time to functional recovery, and complications related to treatment., Results: Complete radiologic healing of the lesion (mean, 15.3 months) and functional recovery (mean, 5.6 months) were observed in all patients treated with either approach. No significant differences were noted in the time to complete radiologic healing or the time to functional recovery between the two groups. There were no recurrences with either approach until the last follow-up (mean, 56 months). Complications were common with anti-cancer chemotherapy, but indomethacin was well-tolerated., Conclusions: Indomethacin seems to be effective for treating isolated LCH of bone in children. Hence, morbidities associated with aggressive treatment approaches such as anti-cancer chemotherapy or surgery can be avoided.

We describe a rare case of secondary malignant fibrous histiocytoma (MFH) following Langerhans cell histiocytosis (LCH). A 23-year-old Japanese male exhibited systemic lymphadenopathy, multiple lung tumors, and osteolytic changes in bilateral iliac bones in 1989. A biopsy specimen from the left iliac bone revealed an infiltration of S-100 protein-positive histiocyte-like cells intermingled with eosinophils, which confirmed the diagnosis of eosinophilic granuloma, a type of LCH. Although the patient was treated with prednisolone initially, the disease did not respond well and progressed gradually over time. The patient subsequently received multiple courses of chemotherapy and immunosuppressive therapy with many kinds of anticancer agents for 6 years. He also received radiotherapy totaling 136.8 Gy for lung tumors and osteolytic lesions of the pelvis. In 1997, because of the LCH refractoriness, biopsy was performed again from the right inguinal lymph node. Microscopic examinations demonstrated a mixture of spindle-shaped cells and histiocyte-like cells, which appeared to be in a storiform pattern. The tumor cells were immunohistologically positive for CD68 and vimentin, but negative for CD1a and S-100 protein. Therefore, the patient was diagnosed with MFH. Although chemotherapy was continued, the patient died of pneumonia during the neutropenic period following chemotherapy. Autopsy revealed systemic invasion of MFH and dissemination of mucormycosis. LCH was not detected histologically in any tissues.

Eosinophilic granuloma is characterized by single or multiple skeletal lesions occurring predominantly in children, adolescents, and young adults, it accounts for 70% of Langerhans cell histiocytosis. The common sites are the skull, mandible, ribs, spines, and long bones particularly the femur and the humerus. Thoracic rib involvement is rarely seen. It was often regarded as malignant soft tissue tumor. We report an unusual case of eosinophilic granuloma in a female child presented with a solitary lesion at the thoracic rib.

Purpose: To report the retrospective experience of a reference center in the diagnosis and treatment of orbital eosinophilic granuloma., Methods: A review of the files in the Opththalmic Pathology Laboratory, of the Hospital Universitário Professor Edgard Santos, Universidade Federal da Bahia, between 1974 and 2004, was conducted to identify cases of orbital eosinophilic granuloma. Data of diagnosis and treatment were collected. The hematoxilin and eosin stained sections were reviewed., Results: Three cases with ages of 1, 6 and 11 years were retrieved. In none of them the clinical diagnosis of eosinophilic granuloma was suspected. All patients underwent diagnostic biopsy with characteristic histological aspect. In one case a orbital computed tomography after biopsy exhibited typical findings. All patients improved with systemic steroids./, Conclusions: This is a rare disease, with a difficult clinical diagnosis if radiological evaluation is not available. In spite of its aggressiveness at presentation, the disease shows good response to systemic steroids.

Langerhans cell histiocytosis or histiocytosis X has a variable course from a self-limited eosinophilic granuloma to an aggressive disseminated disease. It mainly affects children. We report a 76-year-old woman with multifocal bone histiocytosis X, involving the rachis, an iliac bone and the skull. The diagnosis has been established by histological exam. Outcome was favourable after chemotherapy.

Eosinophilic granuloma is classified as a Langerhans cell histiocytosis. Although considered a rare pathology, up to 20% of all cases occur in the jaw bones, and radiographically may mimic odontogenic cysts or benign and malignant tumours. Different protocols have been suggested in the literature for treating eosinophilic granuloma. We report a case of polyostotic eosinophilic granuloma in a 34-year-old man showing ill defined mandibular and palatal radiolucencies. Due to the presence of multiple jaw lesions the treatment choice was chemotherapy. The clinical and radiographic feature are described, as well as treatment, complications and patient's follow-up.

Oral eosinophilic granulomas were diagnosed in 16 tigers (Panthera tigris). All lesions were located on the hard or soft palate and typically consisted of flat or slightly raised circular ulcers. Histologic features of these lesions were essentially identical to those seen in oral eosinophilic granulomas of domestic cats and dogs. No clinical signs were noted in eight cases, though various degrees of inappetence, excessive salivation, and dysphagia were noted in the other eight tigers. Six cases were not treated. Treatment for the remaining 10 cases centered on corticosteroids and additional treatments included surgical removal, cryotherapy, antibiotics, and chlorpheniramine. Treatment with corticosteroids did appear to be effective in some cases, though lesions would worsen after cessation of therapy and no cases were cured. In addition, three cases developed complications possibly related to this corticosteroid therapy. The etiology of these lesions remains unknown, though an underlying allergic condition is likely.

Langerhans cell histiocytosis (LCH) is a histiocytic disorder in which the Langerhans cell is arrested in a premature, partially active state. The disease comprises a spectrum of disorders, the most benign of which is a single-system unifocal disease. Lesions in single-system LCH are typically limited to the bone. We report a case of a 66-year-old man with a single-system disease limited to the skin, who was successfully treated with intralesional triamcinolone. This minimally invasive treatment method warrants further studies of intralesional corticosteroid injection for skin lesions of LCH.

A case of solitary eosinophilic granuloma (EG) of the brain in a 53-year-old woman is presented. The tumor developed in the anterior cranial fossa causing destruction of the skull basis and the retrobulbar space, accompanied by vision impairment. The tumor was partially excised and the patient was irradiated postoperatively with 40 Gy 6 MV x-rays, followed by 10 cycles of chemotherapy. Follow-up computed tomography (CT) showed no response at 30 Gy and no progression during chemotherapy. Attention is drawn to cases of EG indolent in growth but resistant to radiation and chemotherapy.

Eosinophilic granuloma complex (EGC) is a syndrome occurring in cats, characterized by lesions affecting the skin and the oral cavity. Conventional treatment is mainly symptomatic and may have undesirable side effects. This paper summarizes homeopathic treatment with snake remedies of cats suffering from EGC. Snake remedies were chosen by individual repertorizations and administered in different dilutions. Reactions were mostly quick, leading to significant improvements, including complete recoveries.

Objectives: To report a patient with a rare case of orbital eosinophilic angiocentric fibrosis (EAF) and to review the literature., Design: Interventional case report., Methods: A 61-year-old man presented with a 6-week history of right periorbital edema and painless proptosis. Examination revealed a nonaxial proptosis, lateral globe displacement, and mild limitation in right eye adduction., Main Outcome Measures: Clinical course and radiological and histological findings., Results: Orbital imaging revealed a right medial orbital mass with involvement of middle ethmoidal air cells. An orbital biopsy of the mass demonstrated an inflammatory infiltrate with a marked eosinophilic component, onion skinning of vessels, and surrounding fibrosis. The diagnosis of orbital EAF was made. There was no response to a 3-month treatment course with systemic steroids, but the patient did not want any further surgical interventions., Conclusion: Although orbital EAF is rare, ophthalmologists need to be aware of this entity, as it may invade the orbit from the sinonasal tract or present as a localized orbital mass. The presence of even minimal sinus involvement and the characteristic histopathology are useful in establishing the correct diagnosis.

Canine eosinophilic granuloma (CEG) is a rare skin disease of unknown origin. It has been reported in Siberian huskies, Cavalier King Charles spaniels and occasionally in other breeds. The lesions comprise nodules or plaques, mostly localised in the oral cavity. A case of a single cutaneous nodular lesion of CEG on the eyelid of a Siberian husky is described. Complete remission was achieved with oral glucocorticoid treatment.

Purpose: To determine the association of antineutrophil cytoplasmic antibodies (ANCA) and leukotriene receptor antagonists with disease activity in a large series of patients with Churg-Strauss syndrome., Methods: Potential subjects were identified by a computerized search of the Mayo Clinic Rochester database for the years 1990 to 2000. Patients meeting one of three classification schemes for Churg-Strauss syndrome were included., Results: Ninety-one patients met the inclusion criteria. Clinical manifestations were similar to those in previous reports. Mortality was similar to that in the general population. ANCA testing was performed in 74 patients. Seventy-three percent (n = 22) of the 30 patients tested before therapy were ANCA positive, as were 75% (n = 12) of the 16 patients tested during a disease flare. In comparison, 16% (n = 8) of the 49 tested during remission were ANCA positive. Serial measurements indicated a correlation of ANCA levels with disease activity. Central nervous system involvement was the only clinical manifestation that correlated with ANCA status (P = 0.05). Twenty-three patients received leukotriene receptor antagonists, of whom 16 (70%) began treatment before diagnosis and 6 (27%) began during remission. Two of those treated after diagnosis relapsed. In 1 patient the relation between disease and leukotriene receptor antagonist use could not be determined. Use of leukotriene receptor antagonists did not affect the time between onset of asthma and manifestations of vasculitis, and was not correlated with organ manifestations, except sinus disease., Conclusion: No one classification scheme identified all patients. Churg-Strauss syndrome has a better prognosis than other ANCA-associated vasculitides. ANCA status correlates with disease activity, whereas a pathogenic role for leukotriene receptor antagonists in the development of Churg-Strauss syndrome was not noted.

Background: Eosinophilic granuloma, Hand-Schüller-Christian disease an Letterer-Siwe disease are characterised by ideopathic proliferation of histiocytes producing focal or systemic manifestations. Definitive diagnosis of histiocytosis is made by histopathology including immunohistochemical detection of S-100 and CD1a antigens. In general these diseases are summarised under the term Langerhans-cell histiocytosis (LCH). The localised form of LCH, in which the disease is limited to bones, lymphatic nodes or the lung, is commonly referred to as eosinophilic granuloma. Surgical excision, radiotherapy and chemotherapy, either alone or in combination, are the main treatment options., Case Report: We present the case of a nine-year old boy with an extended eosinophilic granuloma of left temporal bone. The patient was submitted to a chemotherapeutic protocol with glucocorticoids, vinblastine and etoposide. Until today a successful treatment and a complete remission for one year can be reported., Conclusions: Unifocal eosinophilic granuloma is usually treated by local excision and low-dose irradiation. However treatment with the chemotherapeutic protocol offered an excellent alternative avoiding extensive surgical destruction of the temporal bone in this case.

Purpose: To describe the systemic treatment of recurrent eosinophilic granuloma., Methods: Case report and review of Langerhans cell histiocytosis., Results: A 9-year-old boy had painful, right upper eyelid swelling. A computed tomography scan showed a right superolateral orbital mass with evidence of bony erosion. Frozen section evaluation was consistent with eosinophilic granuloma. Biopsy and curettage were performed. Systemic work-up was negative for extraorbital disease. Recurrence of the orbital mass 6 weeks after surgery was confirmed by magnetic resonance imaging (MRI). Four weeks after treatment with prednisone and vinblastine, MRI showed a marked decrease in the size of the orbital lesion. Additional treatment with 6-mercaptopurine and methotrexate was initiated, and MRI 6 months later showed complete resolution of the lesion., Conclusions: The treatment of recurrent eosinophilic granuloma is challenging. The goal is to effectively cure the lesion with minimal short-term and long-term side effects. Systemic steroids, vinca alkaloids, antimetabolites, and antifols are preferred because they have less toxicity and mutagenicity. Combination chemotherapy used in conjunction with treatment by a pediatric hematologist/oncologist appears to be an effective method of treating recurrent disease.

Purpose: To describe the findings and outcomes in eosinophilic granuloma (unifocal Langerhans cell histiocytosis [LCH]) of the orbit, and to explain the paradox of aggressive bone destruction responsive to minimal intervention., Methods: Retrospective, consecutive, interventional case series of patients treated from 1985 through 2001. Minimum inclusion criteria were demonstration of CD1a positivity or Birbeck granules, treatment by a single surgeon, systemic evaluation by a pediatric oncologist, and follow-up of 12 months. A pathogenetic construct was assembled from general LCH concepts and the specific orbital findings., Results: Seven patients met study criteria. All were male, 2 to 16 years of age. All had eyelid or forehead swelling and osteolytic defects, with symptoms of 2 to 6 weeks' duration. All underwent incisional biopsy, with frozen-section examination suggestive of LCH in 6 of 7 cases. The 2 earliest patients received low-dose irradiation after simple biopsy. The 5 most recent patients had subtotal curettage at the time of biopsy; 4 of 5 received simultaneous intralesional corticosteroid injection. In all cases, systemic evaluation showed no other focus of LCH, reossification was timely, and no local recurrence or additional focus was noted in follow-up of 1 to 17 years., Conclusions: Transient immune dysfunction may provoke the cytokine-mediated proliferation of pathologic Langerhans cells within the hematopoietic marrow of the anterolateral frontal bone. These cells cause osteolysis through elaboration of interleukin-1 and prostaglandin E2. Corticosteroids can inhibit the mediators. We recommend incisional biopsy, frozen-section provisional diagnosis, subtotal curettage, intralesional corticosteroid instillation, postoperative systemic evaluation, and long-term follow-up.