1/6. hypereosinophilic syndrome presenting as cutaneous necrotizing eosinophilic vasculitis and Raynaud's phenomenon complicated by digital gangrene.Cutaneous necrotizing eosinophilic vasculitis is a recently identified type of vasculitis that is characterized by an eosinophil-predominant necrotizing vasculitis affecting small dermal vessels. Clinically, it presents with pruritic erythematous and purpuric papules and plaques, peripheral eosinophilia and a good response to systemic steroid therapy. This vasculitis can be idiopathic or associated with connective tissue diseases. Although the pathogenic roles of eosinophil-derived granule proteins and interleukins have been documented in diseases associated with eosinophilia, a role of CD40 (a glycoprotein of the tumour necrosis factor receptor superfamily) has rarely been described. We describe two patients with idiopathic hypereosinophilic syndrome (HES) presenting with multiple erythematous patches and plaques on the lower extremities and Raynaud's phenomenon. They satisfied the criteria for the diagnosis of HES by clinical and laboratory investigations. Histopathology of the cutaneous lesions revealed prominent eosinophilic infiltration with local fibrinoid change in vessel walls in the dermis and subcutis. Immunohistochemical detection of CD3, CD4, CD8 and CD40 was performed. Infiltrating eosinophils were strongly stained by anti-CD40 monoclonal antibody. One patient improved with prednisolone, pentoxifylline and nifedipine, without recurrence. The other patient initially improved with steroids, but after self-withdrawal of steroid developed digital ischaemia that evolved to severe necrosis and required amputation. Cutaneous necrotizing eosinophilic vasculitis, Raynaud's phenomenon and digital gangrene may develop as cutaneous manifestations of HES. CD40 may play a part in the pathogenesis of eosinophilic vasculitis in HES.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/6. Necrotic cutaneous lesions induced by hypereosinophilic syndrome secondary to a T-cell lymphoma.Cutaneous lesions of hypereosinophilic syndrome (HES) may display various presentations. These are important to recognize to allow early diagnosis. We report an unusual case of a young man with HES secondary to a splenic T-cell lymphoma, revealed by diffuse necrotic cutaneous lesions. Later on, brain and heart infarctions developed, leading to the patient's death. Analysis of skin biopsy specimens showed occluded dermal vessels surrounded by activated eosinophils. Because a complete analysis of hemostasis was unremarkable, these findings suggest that dermal vessel thrombosis was the direct consequence of the migration of activated eosinophils. Cutaneous lesions of HES are protean and probably reflect the differences between the lesional events induced in situ by activated eosinophils.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/6. Fatal fulminant hypereosinophilic syndrome with a petechial rash in a 16-year-old girl.Peripheral blood eosinophilia and eosinophilic tissue infiltration characterize the hypereosinophilic syndrome, which if untreated can be fatal. Its manifestations are protean. Often a diagnosis of exclusion, it presents diagnostic challenges. A fatal case of hypereosinophilic syndrome in a 16-year-old girl with petechiae, edema, urticaria, and diffuse erythema unfolded over 2 weeks. The histopathology of the petechiae demonstrated an eosinophilic and mixed cell interstitial and perivascular infiltrate and microthrombi in vessels. There was no evidence of T-cell clonality. Despite treatment with cyclosporine and methylprednisolone, she died. The age group; fulminant, fatal course without neoplasia; striking eosinophilia; and petechial rash are notable.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
4/6. Sudden unexpected death in childhood due to eosinophilic myocarditis.A 12-year-old boy with no previous serious medical history experienced abdominal discomfort and chest pains for 5 days and suddenly died. The autopsy revealed diffuse and extensive infiltration of eosinophils into the myocardium, with poorly formed granulomas and few fibrotic changes. The necrotic changes was so extensive that Charcot-Leyden crystals formed. The other visceral organs had no specific pathologic changes except for mild lymphocytic infiltration with an increase in goblet cells in the bronchial areas and eosinocytosis in the blood vessels. An initial viral infection seemed to have caused subsequent eosinophil activation due to an allergic condition. Eosinophilic myocarditis is a rare cause of sudden death in apparently healthy children. Cardiac toxicity of eosinophils is, however, well established and dominates the ultimate prognosis of patients with complicated eosinophilia.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
5/6. basement membrane thickening in small intramyocardial vessels in eosinophilic myocarditis.The case reported in this paper, besides illustrating several aspects of the eosinophilic myocarditis, documents a yet undescribed feature of the cardiac involvement in idiopathic hypereosinophilic syndrome characterized by marked basement membrane thickening of small intramyocardial vessels. We are unaware of previous reports showing the presence of an extensive thickening of the basement membrane of arteriolar and capillary intramyocardial vessels in eosinophilic myocarditis. Considering the findings in the present case, i.e., the focal nature of the lesions, the type of necrosis, and the diffuse microangiopathy, it can be hypothesized that the microvascular disease could play a role in the development of the myocardial changes.- - - - - - - - - - ranking = 3keywords = vessel (Clic here for more details about this article) |
6/6. hypereosinophilic syndrome and myocardial infarction in a 15-year-old.The hypereosinophilic syndrome (HES) is a rare yet frequently fatal disorder of unknown etiology characterized by markedly elevated eosinophil counts and subsequent multiple organ failure due presumably to eosinophil-derived protein toxicity. We describe the laboratory and anatomic findings in a 15-year-old female with extraordinarily high circulating levels of eosinophil major basic protein (MBP) who sustained a precipitous cardiac death secondary to a massive myocardial infarction. Postmortem examination showed marked cardiomegaly with extensive recent left ventricular infarction. Occlusive thrombosis of small blood vessels was evident in the myocardium, spleen, lungs, and kidneys. Immunofluorescent staining showed massive MBP deposition in multiple organ parenchyma including the heart, renal glomeruli, adrenal cortex, bronchioles, and other visceral organs, suggesting a causal relationship. We hypothesize on the mechanisms of eosinophil toxicity in HES.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |