Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Eosinophil cationic protein (ECP) is exclusively secreted only by the eosinophilic leukocyte. In this study the ECP concentration in the serum was measured in patients (n = 155) with various skin disorders and compared with the number of circulating eosinophils. The presence of activated eosinophils in the skin was also studied immunohistochemically using the monoclonal antibody EG-2, which recognizes both the eosinophil protein X (EPX/EDN) and ECP. EG-2 distinctly revealed these proteins in the eosinophils and their granules. Non-activated eosinophils were studied with the monoclonal antibody EG-1. In most cases this did not disclose any more eosinophils and often it was located more diffusely and not seldom on collagen fibers. Elevated serum ECP but normal numbers of circulating eosinophils were found in half of the patients with progressive plaque psoriasis and long-standing daily chronic urticaria. In patients with prurigo nodularis, papular erythematous eruptions, vasculitis, purpura and toxic drug reactions, Wells' syndrome, porphyria cutanea tarda and persistent light reaction the serum ECP was increased, although in some cases the number of circulating eosinophils was normal. In these disorders an increased number of activated eosinophils was found in the skin. Both serum ECP and the number of activated eosinophils normalized when the patients' condition improved. In atopic dermatitis the serum ECP and the number of activated eosinophils in the skin were increased only during exacerbation of the disease. High serum levels of ECP and activated eosinophils in the skin are frequent findings in many skin disorders in spite of often normal blood eosinophil counts.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Eosinophilic cationic protein (ECP) in skin disorders. 168 31

This report describes a 40-year-old man suffering from Well's syndrome (recurrent granulomatous dermatitis with eosinophilia) who presented an anterior myocardial infarction complicated by shock and 3rd degree A-V block. The patient died within 12 hours of admission to the hospital. At autopsy, both main coronary arteries showed proximal aneurysms occluded by thrombi. On light microscopy, the aneurysmatic coronary walls were infiltrated by numerous eosinophils, lymphocytes and plasma cells. Similar cellulitis, mainly perivascular, was found in kidneys and anterior mediastinum. Because the patient had been treated with large doses of diclofenac and piroxicam owing to painful arthralgias, the Authors discuss the possible allergic pathogenesis of the vasculitis.
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PMID:[Well's syndrome with eosinophilic vasculitis, coronary aneurysms and myocardial infarction]. 261 11

Wells' syndrome is a distinctive dermatosis clinically resembling acute cellulitis with solid edema; it resolves spontaneously after weeks or months without residues. Recurrences over many years are common. Light microscopy is characteristic for the disease, with diffuse tissue eosinophilia and marked edema, fibrinoid "flame figures," and palisading microgranuloma. Vasculitis is never found. Eosinophilia of the peripheral blood is a frequent feature. Etiology and pathogenesis are unknown, but the disease has been found to be associated with hematologic disorders in several cases, and recurrences can often be related to infections, arthropod bites, drug administration, or surgery. The diagnosis of Wells' syndrome should be based on the typical clinical picture and the course of the disease with its recurrences and histopathology. Flame figures in histologic sections are an important diagnostic feature but not diagnostic per se for the disease because they represent a reaction pattern that can occur in other conditions. A dilution of Wells' syndrome by making flame figures the central criterion of diagnosis and by lumping all flame figure-positive skin reactions together is therefore unjustified.
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PMID:Wells' syndrome is a distinctive disease entity and not a histologic diagnosis. 291 71

Among the dermatoses associated with tissue eosinophilia are pemphigus vegetans, bullous pemphigoid, granuloma facial and Wells' syndrome. Eosinophilic spongiosis can occur in the early stage of pemphigus. Pemphigus herpetiformis, a recently introduced term, does not represent a well defined entity but develops into either pemphigus vulgaris or pemphigus foliaceus. Granuloma facial is basically vasculitis. Eosinophilic cellulitis or Wells' syndrome shows numerous eosinophils which degranulate and through confluence of their granules form the so-called flame figures.
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PMID:[Inflammatory skin diseases and hypereosinophilia]. 330 10

Study of 18 patients with biopsy diagnoses of eosinophilic panniculitis revealed diverse patterns of systemic disease, including Wells' syndrome, vasculitis, atopy, and erythema nodosum as well as localized panniculitis. Significant associated diseases included psychiatric illness, 6 (drug dependency, 4); atopy, 5 (asthma, 3); malignancies, 5; immune complex vasculitis, 4; thyroid disease, 2; Wells' eosinophilic cellulitis, 2; glomerulonephritis and sarcoidosis, 1 each. The skin lesions varied from urticarial papules and plaques to purpura, pustules, and ulcerative lesions but always included a nodular subcutaneous component, frequently as a presenting complaint. Eosinophilic panniculitis is a non-specific finding that can signify localized disease, such as an insect bite or injection lipophagic granuloma in a drug-dependent patient, or systemic lymphoma or immune reactive disease. Eosinophilic panniculitis in erythema nodosum is perhaps its most confusing presentation.
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PMID:Eosinophilic panniculitis: a clinicopathologic study. 370 Jul 70

Granulomatous dermatitis with eosinophilia (Well's syndrome) occurred in an 11-year-old boy. To our knowledge, eosinophilic cellulitis in a child in whom atrophic alopecia of the affect scalp later develops during the regression phase has not previously been reported. No notable effect of steroid therapy was observed. The cause of eosinophilic cellulitis is still unknown, but the presence of disseminated fibrinoid necrosis, vasculitis, eosinophilia, and a positive antinuclear factor test result might indicate an autoimmune or allergic disease.
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PMID:Eosinophilic cellulitis. (Well's syndrome) in a child. 725 22

Between 1981 and 1991, seventy-two cases of brain stem lesions have undergone CT or MRI-directed stereotactic surgery using the Brown-Roberts-Wells or Cosman-Roberts-Wells stereotactic systems. In 16 cases, the pre-operative diagnosis of haematoma had been made and the procedure was for therapeutic aspiration. In fifty-six cases, the pre-operative diagnosis was uncertain although intrinsic tumour was suspected. Histological diagnosis was obtained in fifty-four of these and unexpected findings of haematomas, granulomas, leucoencephalopathy, demyelinating disease, vasculitis and radiation necrosis occurred in over 15% of the cases. There were no operative deaths and morbidity was low. Advances in neuroradiological imaging with CT and MRI have increased the detection rate of brain stem lesions and image-directed stereotactic biopsy makes it possible in most cases to obtain histological diagnosis with relatively low morbidity which is important for treatment and prognosis. MRI-directed biopsies can compliment CT-guided biopsies in most cases where the lesion is not adequately delineated on the CT image.
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PMID:Stereotactic management of intrinsic brain stem lesions. 821 98

We report a patient with Churg-Strauss syndrome (CSS) with asthma, eosinophilia, nasal polyposis and ANCA-associated multisystem vasculitis, who's skin eruption started with erythematous urticarial-plaques followed by haemorrhagic bullae. Histology of the plaques revealed 'flame figures' in the dermis with no granulomatous or vasculitic process, consistent with the diagnosis of eosinophilic cellulitis or Wells' syndrome. The association of CSS and Wells' syndrome observed in this patient may have a common pathogenesis. CSS may induce Wells' syndrome by an unknown factor.
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PMID:Bullous eosinophilic cellulitis (Wells' syndrome) associated with Churg-Strauss syndrome. 1260 82

A collagenolytic or necrobiotic non-infectious granuloma is one in which a granulomatous infiltrate develops around a central area of altered collagen and elastic fibers. The altered fibers lose their distinct boundaries and exhibit new staining patterns, becoming either more basophilic or eosinophilic. Within the area of altered collagen, there may be deposition of acellular substances such as mucin (blue) or fibrin (red), or there may be neutrophils with nuclear dust (blue), eosinophils (red), or flame figures (red). These color distinctions can be used as a simple algorithm for the diagnosis of collagenolytic granulomas, i.e. "blue" granulomas vs. "red" granulomas. Eight diagnoses are included within these two groupings, which are discussed in this two-part article. In this first part, the clinical presentation, pathogenesis, and histologic features of the "blue" collagenolytic granulomas are discussed. These are the lesions of granuloma annulare, Wegener's granulomatosis, and rheumatoid vasculitis. In the subsequent half of this two-part series, the "red" collagenolytic granulomas will be discussed; these are the lesions of necrobiosis lipoidica, necrobiotic xanthogranuloma, rheumatoid nodules, Churg-Strauss syndrome, and eosinophilic cellulitis (Well's syndrome).
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PMID:Collagenolytic (necrobiotic) granulomas: part 1--the "blue" granulomas. 1505 19

Wells syndrome, also known as eosinophilic cellulitis, is an uncommon condition whose etiology often remains a mystery. Patients present with recurrent cutaneous swellings that are often cellulitic in appearance. Histopathologic evaluation of the skin lesions reveals a dense dermal eosinophilic infiltrate, marked edema, and characteristic "flame figures". Notably, the picture is devoid of vasculitis. Therapy with low-dose systemic steroids has proven variably successful. Clinical evidence lending support for the efficacy of other medications has been, for the most part, anecdotal. We present a case of Wells syndrome, review the literature, and discuss therapeutic options.
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PMID:Wells syndrome: an enigmatic and therapeutically challenging disease. 1703 61


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