Eosinophilic Vasculitis Research Articles

Cutaneous Vasculitis Update: Neutrophilic Muscular Vessel and Eosinophilic, Granulomatous, and Lymphocytic Vasculitis Syndromes

Most biopsies of cutaneous vasculitis will exhibit a small vessel neutrophilic vasculitis [leukocytoclastic vasculitis (LCV)] that is associated with immune complexes on direct immunofluorescence examination or, less commonly, antineutrophilic cytoplasmic antibodies (ANCA) by indirect immunofluorescence testing. Is in uncommon for skin biopsy to reveal solely a neutrophilic arteritis signifying the presence of cutaneous polyarteritis nodosa or, if accompanied by significant lobular panniculitis, nodular vasculitis/erythema induratum. In other cases, cutaneous vascular damage (fibrinoid necrosis, muscular vessel wall disruption, or endarteritis obliterans) will be mediated by a nonneutrophilic inflammatory infiltrate. Eosinophilic vasculitis can be a primary (idiopathic) process that overlaps with hypereosinophilic syndrome, or it can be a secondary vasculitis associated with connective tissue disease or parasite infestation. Authentic cutaneous granulomatous vasculitis (versus vasculitis with extravascular granulomas) can represent a cutaneous manifestation of giant cell arteritis, an eruption secondary to systemic disease such as Crohn's disease or sarcoidosis, or a localized disorder, often a post-herpes zoster (HZ) phenomenon. Lymphocytic vasculitis is a histologic reaction pattern that correlates with broad clinical differential diagnosis, which includes connective tissue disease - mostly systemic lupus erythematosus (SLE), endothelial infection by Rickettsia and viruses, idiopathic lichenoid dermatoses such as perniosis or ulcerative necrotic Mucha-Habermann disease, and angiocentric cutaneous T-cell lymphomas. Skin biopsy extending into the subcutis, identifying the dominant inflammatory cell and caliber of vessels affected, extravascular histologic clues such as presence of lichenoid dermatitis or panniculitis, and correlation with clinical data allows for accurate diagnosis of these uncommon vasculitic entities.

Role of cysteinyl-leukotrienes in the pathogenesis of nasal polyposis in patients with asthma

Cysteinyl leukotrienes (CysLTs: leukotrienes C4, D4, and E4) have long been implicated in the pathogenesis of allergic diseases such as asthma. CysLTs are potent bronchoconstrictors that have the additional effects of edema, mucous secretion, and eosinophilic accumulation, and airway remodeling. LTE4 has been identified as a major metabolite of LTC4, and urinary LTE4 (U-LTE4) is considered as the most reliable analytic parameter for monitoring the endogenous synthesis of CysLTs. From our extensive study of U-LTE4 in adult asthma, we identified four factors for hyperleukotrienuria. These factors were aspirin-intolerance, eosinophilic nasal polyposis (ENP), vasculitis, and severe asthma. In ENP there is prominent infiltration of eosinophils in the sinus and polyp tissues, which is linked to adult asthma and aspirin-sensitivity, and ENP is the most important factor for overproduction of CysLTs in asthmatics. We demonstrated that there is a significant decrease in the U-LTE4 concentration after the sinus surgery of asthmatics with and without aspirin intolerance. Recent studies reported that ENP tissue contain and produce a large amount of CysLTs, and there is a close relationship between CysLT production and eosinophil accumulation in ENP. These observations suggest that ENP is not only a local allergic disease, but also a systemic inflammatory disease with CysLT overproduction.

Eosinophilic vasculitis: A Rare Presentation of Whipple’s Disease

Whipple's disease is a multisystem infectious disease caused by the bacterium Tropheryma whippelii. A case with an unusual presentation is reported. A 66-year-old man presented with a febrile vasculitic rash on his forearms. An extensive rheumatological, hematological and infectious workup gave negative results, apart from mild anemia and eosinophilia. An abdominal computed tomography revealed a retroperitoneal lymphadenopathy, and a skin biopsy revealed an eosinophilic vasculitis. This diverted the work toward ruling out a lymphoma or a vasculitic process. A lymph node biopsy was then performed and showed a diffuse neutrophilic inflammation with abundant foamy macrophages, fat necrosis and lipogranuloma formation. These findings were considered to be nonspecific and no further pathological investigation was carried out. After a course of corticosteroids, diarrhea and weight loss predominated and subsequently a diagnosis of Whipple's disease was confirmed on a small-bowel biopsy. Lymph node involvement was then confirmed on re-evaluation using the appropriate stains.

CHURG-STRAUSS SYNDROME IN A 12-YEAR-OLD ASTHMATIC GIRL

PURPOSE: Severe asthma with rhinosinusitis and peripheral eosinophilia is not unusual in children. These children have eosinophilia on the basis of atopic disease and have positive skin tests and elevated IgE levels. In the absence of atopy, progression to pulmonary and/or systemic eosinophilic vasculitis (Churg-Strauss Syndrome) is often seen in adults but rarely in children. We report such a case.

Churg-Strauss Syndrome in Children: A Clinical and Pathologic Review

Churg-Strauss syndrome is a vasculitis accompanied by asthma and eosinophilia. It is generally considered a disease of adults; occurrence in children has been reported infrequently. Here we report 2 pediatric patients with Churg-Strauss syndrome manifesting with prominent pulmonary involvement. One, a 16-year-old with a previous history of asthma, presented with pleuritic chest pain and a peripheral pulmonary nodule complicated by an eosinophilic pleural effusion. The other patient presented at age 6 with cough, weight loss, and radiographic infiltrates. Lung biopsies revealed elements characteristic of Churg-Strauss syndrome, including eosinophilic microabscesses and vasculitis. Three- and 5-year follow-up showed continued symptoms in both patients despite medical therapy. Both patients illustrate many of the typical features of Churg-Strauss syndrome. We report these cases to expand the scant knowledge about Churg-Strauss syndrome in pediatric patients and to heighten awareness that this serious disease may affect the pediatric population. The relevant literature on Churg-Strauss syndrome, with specific reference to childhood cases, is reviewed.

Atypical eosinophilic angiocentric fibrosis on nasal septum

Eosinophilic angiocentric fibrosis (EAF) is a rare benign condition of unknown aetiology and is most commonly found in the nasal septum and sinus mucosa. We report a case of EAF and present a review of the available literature. A 51-year-old man with progressive nasal obstruction was referred to our hospital. CT and MRI scans revealed a mass on the nasal septum; this was surgically excised. Histological analysis of the resected tumour showed an inflammatory infiltrate with a predominance of eosinophils present in fibrous matrix and absence of eosinophilic vasculitis and onion skinning. Histologically, it resembled granuloma faciale. However, our case was considered to be an EAF although eosinophilic vasculitis and onion skinning were not observed. This was because a cutaneous lesion was absent and the lesion was limited to the nasal septal mucosa.

Isolated eosinophilic mesenteric vasculitis with extensive thrombosis and splenic infarction in a 13-year-old boy

There are no generally accepted diagnostic criteria for primary systemic vasculitis, and the application of classification as diagnostic criteria is not feasible and may even be misleading. We report a case of a 13-year-old boy with acute abdomen who was found to have isolated eosinophilic mesenteric vasculitis with extensive thrombosis and splenic infarction. All serological tests were negative, including antineutrophil cytoplasmic antibody. The vasculitis had been successfully controlled with surgical intervention, steroid, and cyclophosphamide therapy. This may be an atypical presentation of Churg-Strauss syndrome.

Angiostrongylus vasorum Infection in a Coyote (Canis latrans) from Newfoundland and Labrador, Canada

Tissue samples and feces were collected from a dead, adult female coyote (Canis latrans) found at the side of the road in late March 2003 in the Avalon Peninsula region of Newfoundland, Canada. The coyote apparently died of vehicular-related trauma. Samples of lung, brain, heart, liver, and kidney were fixed in formalin and submitted for histologic examination. The entire remaining lung and heart also were submitted for examination. The coyote was diagnosed with moderate, multifocal, granulomatous interstitial pneumonia with eosinophilic vasculitis and many intralesional nematode eggs, larvae, and occasional intravascular adult worms. Adult nematodes recovered from the pulmonary arteries were identified as Angiostrongylus vasorum. Small foci of granulomatous inflammation, often containing nematode eggs and larvae, were scattered in the brain and kidney. To our knowledge, this is the first report of A. vasorum infection in a coyote from the only endemic area of infection in North America.

Recurrent cutaneous eosinophilic vasculitis presenting as annular urticarial plaques.

Sir, Clinical and histological cutaneous necrotizing vasculitis of dermal small vessels with an almost exclusively eosinophilic infiltration and without any features of the systemic disease has recently been described by Chen et al. as recurrent cutaneous eosinophilic vasculitis (RCEV) (1, 2). This disease is rare and only a few cases have been reported (1–4). We here describe one more case of RCEV presenting as annular urticarial plaques.

Carbimazole induced eosinophilic granulomatous vasculitis localized to the stomach

Vasculitis associated with anti-neutrophil cytoplasmic antibodies has been reported in patients treated with anti-thyroid drugs and especially propylthiouracil. We report here a case of granulomatous eosinophilic vasculitis mimicking gastric neoplasm and peripheral eosinophilia in a 27-year-old man who was treated with carbimazole for 5 months for Graves' disease. Clinical and morphologic features resolved within 2 months after stopping the drug, suggesting a causative role for the drug. To our knowledge, this is the first biopsy-proven granulomatous eosinophilic vasculitis associated with this drug.

Response to Imatinib Mesylate in a Patient with Idiopathic Hypereosinophilic Syndrome Associated with Cyclic Eosinophil Oscillations

A 26-year-old man with idiopathic hypereosinophilic syndrome (HES) was treated with imatinib mesylate following a 5-year history of prednisolone therapy. The patient had hypereosinophilia (absolute eosinophil counts >1500/microL) occurring in cyclic oscillations as well as histologically diagnosed eosinophilic vasculitis, bursitis, and periodic soft-tissue swellings. Laboratory data revealed high levels of serum tryptase and increased numbers of mast cells in the bone marrow, but serum interleukin 5 levels were within the normal range. The disease initially responded well to 100 mg/day of imatinib mesylate but recurred 8 weeks later. Thereafter, a daily 200-mg dose was temporarily effective. Despite the response to imatinib, the FIP1L1-PDGFRA fusion gene was not detected by fluorescence in situ hybridization analysis. Additional molecular and cytogenetic studies showed neither translocations of platelet-derived growth factor receptor (PDGFR) genes nor mutations in the c-KIT or the PDGFR genes. Although imatinib mesylate is a choice of treatment for patients with HES, its precise molecular mechanism in individual cases remains to be clarified.

Hypereosinophilia Presenting as Eosinophilic Vasculitis and Multiple Peripheral Artery Occlusions without Organ Involvement

We report here a case with hypereosinophilia and peripheral artery occlusion. A 32-yr-old Korean woman presented to us with lower extremity swelling and pain. Angiography revealed that multiple lower extremity arteries were occlusive. The biopsy specimen showed perivascular and periadnexal dense eosinophilic infiltration in dermis and subcutaneous adipose tissue. Laboratory investigations revealed a persistent hypereosinophilia. She was prescribed prednisolone 60 mg daily. Her skin lesion and pain were improved and the eosinophil count was dramatically decreased. After discharge, eosinophil count gradually increased again. Cyanosis and pain of her fingers recurred. She had been treated with cyclophosphamide pulse therapy. Her eosinophilia was decreased, but the cyanosis and tingling sense were progressive. The extremity arterial stenoses were slightly progressed. Skin biopsy showed perivascular eosinophilic infiltration in the dermis and CD40 ligand (CD40L) positive eosinophilic infiltration. The serum TNF-α was markedly increased. These results suggest that CD40L (a member of TNF-α superfamily) could play a role in the inflammatory processes when eosinophil infiltration and activation are observed. We prescribed prednisolone, cyclophosphamide, clopidogrel, cilostazol, beraprost and nifedipine, and she was discharged.

Churg-Strauss Syndrome: A casereport

Churg-Strauss syndrome (CSS) is a rare systemic vasculitis of unknown cause, occurring in patients with asthma and accompanied by hypereosinophilia. Pathologically, CSS is characterized by necrotising eosinophilic vasculitis which may involve multiple organ systems, commonly including the lungs, skin, and peripheral nervous system. Corticosteroids are the first-line therapy, with cyclophosphamide as adjuvant therapy. The overall prognosis is good. We report a case of CSS with the clinical pattern of asthma, hypereosinophlilia (＞10%), pulmonary infiltration, and mononeuritis multiplex. Progressive weakness and numbness in the bilateral hands and lower limbs, with associated walking inability, remained a concern despite steroid treatment. The patient underwent rehabilitation therapy including strengthening exercises, balance training, ambulation training, activities of daily living (ADL) training, and hand function training. We used a Functional Independence Measure motor score and muscle power as a follow-up index. Five months later, she showed improvement in left ankle dorsiflexion muscle power and was able to walk independently with a simple cane. There was a significant improvement in ADL independence and skill. The intractable neuropathic pain was treated successfully by a topical application of capsaicin. In this article, we review the clinical features, diagnosis criteria, and treatment options of CSS. Atrophy and weakness of the limbs, paresthesia, and neuropathic pain result in the functional impairment of daily living and ambulation disability. Thus, in addition to pharmacological treatment, aggressive rehabilitation intervention is essential to improve the patient's functional independence of daily living and life quality.

Coronary and Valve Angiodysplasia Unmasked by Eosinophilic Vasculitis and Endomyocarditis

A previously unreported case of a complex congenital cardiac anomaly consisting of coronary and valvular angiodysplasia, complicated by an eosinophilic endomyocardial disease, is described. The disorder presented clinically with congestive heart failure, which was characterized by evidence of floating masses causing severe pulmonary stenosis and dysfunction of the mitral and aortic valves. Symptoms of cardiac failure disappeared following pulmonary valve replacement and steroid therapy.

Pathology of Pulmonary Vasculitis

There are three major vasculitis syndromes that affect the lung: Wegener's granulomatosis (WG), Churg-Strauss syndrome (CSS), and microscopic polyangiitis (MPA). The pathology of pulmonary vasculitis is complicated because it requires correlation with clinical, laboratory, and radiological features; there is overlap in some histological features among the vasculitis syndromes; biopsies early in the course of disease or after therapy may show atypical or incomplete histological features; the differential diagnosis is complex and includes infection that should not be treated with corticosteriods or immunosupressive agents; and few pathologists have much experience with these cases. Major histological features of necrosis, granulomatous inflammation, and vasculitis characterize WG. The inflammatory consolidation consists of a mixture of neutrophils, lymphocytes, plasma cells, macrophages, giant cells, and eosinophils. Necrosis may take the form of neutrophil microabscesses or geographic necrosis. Granulomas may take several forms, including scattered or loose clusters of giant cells, palisading histiocytes or giant cells lining the border of geographic necrosis or microabscesses, and palisading microgranulomas. Sarcoidal granulomas are very rare. CSS may show eosinophilic pneumonia, allergic granulomas, and eosinophilic vasculitis. Asthmatic bronchitis may also be present. Biopsies from CSS patients are rare because this syndrome is usually diagnosed clinically. Microscopic polyangiitis demonstrates neutrophilic capillaritis and diffuse alveolar hemorrhage.

Eosinophilic pericardial effusion in Churg–Strauss syndrome

Churg–Strauss syndrome (CSS), characterised by a diffuse eosinophilic vasculitis and asthma, is a rare systemic disease of unknown cause, but an overall good prognosis. Cardiac involvement in CSS includes eosinophilic myocarditis, coronary vasculitis, valvular heart disease, systolic dysfunction, pericarditis and ventricular thrombi. Eosinophilic pericardial effusion has not been reported in CSS.

Gnathostomiasis: clinicopathologic study.

Gnathostomiasis is a systemic parasitic disease that is caused by the ingestion of contaminated raw fish, the intermediate host. Involvement of the skin is a common event, and when it does happen, it can produce a superficial or creeping eruption, pseudofurunculosis, and nodular migratory panniculitis. We carried out a retrospective study of 946 cases of gnathostomiasis; 66 of them had skin biopsies. The diagnosis was made based on clinical and epidemio-logic findings as well as the therapeutic response. The most common skin finding was nodular migratory panniculitis affecting the trunk. Most of the patients were males between 20 and 40 years of age. Histopathologically, we were able to see the larva and make a definitive diagnosis in 15 cases, and in 12 cases, the worm was retrieved during the surgical procedure. In remaining cases, despite of our inability to identify the larva, the histopathologic changes were quite characteristic and included: dermal and hypodermal edema with dense mixed infiltrates composed of eosinophils admixed with lymphocytes and neutrophils, eosinophilic vasculitis, flame figures, areas of necrosis, and hemorrhage. Thus, the presence of these histopathologic features in the context of a clinical picture suggestive of gnathostomiasis allows the pathologist to make the correct diagnosis.

Churg-strauss syndrome in an 8-year-old girl.

Churg-Strauss Syndrome (CSS) is a small vessel granulomatous vasculitis that characteristically affects the middle age group and is extremely rare in children. We describe an 8-year-old girl with a prolonged history of cough and eosinophilia that initially was labeled as pulmonary tuberculosis, but after complementary assays, associated with skin biopsy, eosinophilic fibrinoid vasculitis, CSS was eventually diagnosed. In this case, due to her young age and more prevalence of infectious lung diseases in our area, there was a few months delay for correct diagnosis and treatment. After meeting the CSS criteria the appropriate treatment was started and her condition improved.

Experience of 10 years with Churg-Strauss syndrome: An accompaniment to or a transition from aspirin-induced asthma?

Churg-Strauss syndrome is a rare, idiopathic, eosinophilic vasculitis appearing in concurrence with asthma which is often severe. Aspirin-induced asthma is a special clinical syndrome existing in nearly 10 % of adult asthmatics. After leukotriene antagonists had been marketed there has been marked increase in Churg-Strauss syndrome reports among the patients who had been followed up with asthma. This syndrome seems to be more frequent among the patients with aspirin-induced asthma. The role of leukotriene antagonists on the conversion from aspirin-induced asthma to Churg-Strauss syndrome has aroused attention and been questioned. Here we report 7 cases of Churg-Strauss syndrome where three had aspirin induced asthma which we have diagnosed in the last 10 years and where only one of them seems to be related to antileukotriene drug use.

A Case of Churg-Strauss Syndrome with Bilateral Pleural Effusions

A 26-year-old man with a one-year history of asthma and sinusitis presented with bilateral pleural effusions, patch basilar infiltrates on a chest x-ray and a pericardial effusion on an echocardiogram. The peripheral blood showed marked eosinophilia. An obstructive pattern was also observed during the pulmonary fuction test, which was responsive to bronchodilator inhalation. Nerve conduction studies showed right sural neuropathy. Thoracentesis yielded an acidotic exudative effusion with low glucose, low and eosinophilia. An open lung biopsy revealed an eosinophilic interstitial pneumonitis associated with a necrotizing eosinophilic vasculitis, and granulomatous inflammation foci. In the literature, pleural effusions were reported in 29 percent of Churg-Strauss patients, but the number of effusions was low and their characteristics have not been well described. This report describes the characteristic findings of pleural fluid and its histologic features in a case of classical Churg-Strauss syndrome.