Cases reported "Histiocytosis"

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1/3. Intravascular or intralymphatic histiocytosis associated with rheumatoid arthritis: a report of 4 cases.

    BACKGROUND: Various skin lesions occur in association with rheumatoid arthritis (RA). observation: We report a distinctive skin lesion observed in 4 patients with RA. All patients had RA for many years and developed asymptomatic, irregularly shaped erythema over the swollen elbow joints and the nearby part of the forearm. Histopathologically, all cases showed massive aggregates mainly composed of histiocytes in markedly dilated vessels in the dermis, accompanied by a dermal infiltrate of lymphocytes, plasma cells, neutrophils, or a combination of these. A total of 9 cases, including ours, showing similar histopathologic findings have been reported in the literature, of which 7 were associated with RA and presented relatively common clinical appearance. CONCLUSION: In spite of some disagreement as to whether the dilated vessels are blood vessels or lymphatics, it is most likely that these 7 cases belong to the same clinical entity closely associated with RA.
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2/3. A new variant of mastocytosis: report of three cases clinicopathologically mimicking histiocytic and vasculitic disorders.

    Cutaneous mastocytosis (CM) or urticaria pigmentosa is characterized by abnormal proliferation and accumulation of mast cells. Clinically, CM usually presents as symmetrically distributed red-brown macules or papules that develop weals, erythema and often pruritus on stroking (Darier's sign). The histological hallmark of the disease is an increase in oval to spindle-shaped mast cells in the dermis located around blood vessels and skin appendages. We describe three patients with a new clinicopathological type of CM, which clinically mimics a histiocytic disorder and histologically mimics leucocytoclastic vasculitis (LV). Three infants (two boys and one girl) developed generalized reddish-yellow-brown macules of 3-10 cm with occasional scaling and crusting on the trunk and extremities without further symptoms or organ involvement except variable itching. histology revealed diffuse and dense dermal infiltrates of eosinophils, neutrophils and nuclear debris with perivascular accentuation, imitating LV. This infiltrate masked large epithelioid cells, positive for macrophage markers, which by special histochemical stains for metachromatic granules turned out to be mast cells. This is the first report of this new variant of CM, which may cause considerable diagnostic difficulties both clinically and histopathologically.
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keywords = vessel
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3/3. Recurred Castleman's disease containing a fibrohistiocytic nodular lesion with vascular occlusion.

    A recurring case of Castleman's disease containing a fibrohistiocytic nodular lesion is presented. The recurred tumor was found in the mediastinum of a 40 year old Japanese female who had undergone the extirpation of a mediastinal tumor 18 years previously and had been suffering from rheumatoid arthritis 5 years after the initial removal. Microscopically, the present tumor and the initial removed tumor fulfilled the morphologic criteria for the hyaline-vascular type of Castleman's disease. A well-demarcated nodular lesion (1.5 cm in diameter) was located in the present tumor. It consisted of interlacing oval to short spindle-shaped cells positive for factor xiiia and vimentin, merging with lymphocytes, plasma cells, macrophages and capillaries lined by swollen endothelial cells. Mitotic figures were not noted. Foci of hemorrhage and necrosis were also not detected. The stenosis or occlusion of the large vessels due to intimal fibrosis and medial hypertrophy was observed around the nodular lesion.
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