Cases reported "Skin Diseases, Vascular"

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1/9. angiomatosis of skin with local intravascular immunoglobulin deposits, associated with monoclonal gammopathy. A potential cutaneous marker for B-chronic lymphocytic leukemia. A report of unusual case with immunohistochemical and immunofluorescence correlation and review of the literature.

    Reactive cutaneous vascular proliferation or angiomatosis is associated with various conditions, but is rarely seen secondary to vascular occlusion. We report an unusual case of a 79-year-old female who presented with 8 month history of purpuric facial plaques, with painful crusted ulceration of the nose, later developing similar eruptions on hands, thighs and trunk. Biopsies showed marked angioproliferation, with intravascular (IV) hyaline deposits (PAS , fibrin /-; IgM , fibrinogen , and C3 ), associated with endothelial hyperplasia (factor viii , vimentin ). Immunofluorescence showed IV IgM, fibrinogen, and granular C3 deposits within vessel walls. Initially, extensive investigations only showed minimal monoclonal gammopathy of undetermined significance (MGUS) and repeatedly negative cryoglobulins. After a 3-year follow-up, the patient developed chronic lymphocytic leukemia (B-CLL). This case illustrates a difficult diagnostic challenge. Although this condition resembles other forms of reactive angiomatosis, it shows distinct features and should be considered in the differential diagnosis of unusual vascular proliferations of the skin. The cutaneous lesions are also considered a potential marker for an underlying systemic condition, which may require prolonged clinical follow-up. We believe this condition to be related to other rare cutaneous vascular proliferations associated with plasma cell and lymphoproliferative disorders. Furthermore, we suggest a common pathogenetic pathway resulting from the IV immunoglobulin deposits causing vascular injury, finally leading to the angiomatosis.
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keywords = angiomatosis
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2/9. Eruptive pseudoangiomatosis arising in adulthood: 9 cases.

    Eruptive pseudoangiomatosis has only been reported in children up till now. The purpose of this study is to present the clinical characteristics of eruptive pseudoangiomatosis in adulthood and to discuss the potential role of infectious agents. A retrospective study concerning nine cases of eruptive pseudoangiomatosis observed over a ten year period was undertaken, concerning clinical, histological and epidemiological aspects of the disease. The patients, 8 women and one man had an acute eruption of numerous papules measuring 2-5 mm in diameter, disseminated on the face, limbs and trunk. The papules were erythematous and telangiectatic, surrounded by a white halo. The lesions blanched completely with pressure and refilled from the center on release. Histological examination was performed on 4 patients, showing dilated blood vessels with plump endothelial cells but no evidence of an increased number of vessels. Epidemiological data revealed the onset of the disease during hospitalisation for treatment of cancer or asthma in four patients and among the five remaining patients, two were hospitalized in a retirement home and one was a nurse in a psychiatric hospital. These data suggest that the eruption could be linked with a infectious disease despite the negativity of some serological investigations. We report for the first time cases of pseudoangiomatosis arising in adulthood.
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ranking = 2.6666666666667
keywords = angiomatosis
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3/9. Eruptive pseudoangiomatosis.

    We describe two children with acute onset and spontaneous resolution of angioma-like papules during a viral illness. The biopsy specimens from both patients showed a unique histologic appearance consisting of dilated dermal blood vessels with plump, hobnail-shaped endothelial cells. On the basis of the natural history and the histopathologic features we suggest the diagnosis of eruptive pseudoangiomatosis.
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ranking = 1.6666666666667
keywords = angiomatosis
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4/9. Eruptive pseudoangiomatosis.

    Eruptive pseudoangiomatosis is a rare, benign, spontaneously regressive disease. The term was recently coined to describe a dermatosis characterized by the sudden onset of a few to several bright red angioma-like papules with histopathologic findings distinct from that of true angiomas. We describe a 7-year-old patient with the typical lesions of eruptive pseudoangiomatosis.
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ranking = 2
keywords = angiomatosis
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5/9. Diffuse dermal angiomatosis of the breast.

    BACKGROUND: Diffuse dermal angiomatosis is rare and usually considered a variant of reactive angioendotheliomatosis. It generally involves the extremities of patients with severe vascular disease and other comorbidities. Two patients with breast involvement have been described; however, neither had a relevant medical history or a vaso-occlusive disorder, but both had large pendulous breasts, and 1 was positive for IgM anticardiolipin and antinuclear antibodies. OBSERVATIONS: A 53-year-old woman had a reticulated, erythematous plaque with superficial ulceration and underlying tender nodules on her left breast. She had a history of cardiovascular disease and was a heavy smoker. biopsy of the lesion showed diffuse proliferation of additional endothelial cells and small bland vessels within the papillary and upper reticular dermis. angiography showed almost complete occlusion of the subclavian artery proximally. Diffuse dermal angiomatosis was diagnosed. With isotretinoin therapy, the lesions improved. One month later, after percutaneous subclavian arterial revascularization, the lesion resolved completely. A literature review suggested that a history of heavy smoking, in addition to a history of vascular disease, may be important in the pathogenesis of diffuse dermal angiomatosis. CONCLUSIONS: Clinical acumen is crucial to diagnose diffuse angiomatosis of the breast. Appropriate treatment to alleviate hypoxia may improve the patient's condition.
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ranking = 2.6666666666667
keywords = angiomatosis
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6/9. Diffuse dermal angiomatosis: A previously undescribed pattern of immunoglobulin and complement deposits in two cases.

    Two cases of diffuse dermal angiomatosis are reported in middle-aged women. This rare disease of unknown origin is characterized by increased dermal angiomatosis and ulceration. The clinical and histologic presentations of the presently reported lesions were typical for this disorder. endothelial cells exhibited a normal immunophenotype. The perivascular basement membranes showed a distribution of collagen alpha chains typical for blood vessels, but not for lymphatics. immunohistochemistry revealed other undescribed features. At the site of the clinical lesions, linear and granular deposits of immunoglobulins A and M, and complement were found around the vessels and at the dermal-epidermal junction. The same deposits were also found restricted to the dermal-epidermal junction in the peripheral clinically intact skin. No serological signs of auto-immune disorder were detected in one patient. A monoclonal gammopathy was disclosed in the other patient. A pattern of immunoreactant deposits similar to that disclosed in the two patients was not found in the control specimens, and has not been described so far in other types of vascular hyperplasia and neoplasia. A pathogenic role of these deposits is unsettled and should be further explored.
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ranking = 2
keywords = angiomatosis
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7/9. Eruptive pseudoangiomatosis.

    Eruptive pseudoangiomatosis (EPA) is a rare, self-limiting exanthem, which is known to occur primarily in children. It is characterized by an eruption of distinctive erythematous angioma-like papules often surrounded by a pale halo, with histological findings distinct from that of true angiomas. We describe three women with angioma-like papules. The biopsy specimens from them showed a unique histological appearance consisting of dilated dermal blood vessels with plump endothelial cell and perivascular lymphocytes. On the basis of the clinical and histological findings, we present the adult cases of eruptive pseudoangiomatosis and their etiology.
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ranking = 2
keywords = angiomatosis
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8/9. Eruptive pseudoangiomatosis: a unique childhood exanthem?

    We describe three children with an acute onset and spontaneous resolution of angioma-like papules during an apparent viral illness. A biopsy specimen from one patient revealed a unique histologic appearance that consisted of dilated dermal blood vessels with plump hobnail-shaped endothelial cells. On the basis of the natural history and histopathologic features of this exanthem, we suggest the name eruptive pseudoangiomatosis.
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ranking = 1.6666666666667
keywords = angiomatosis
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9/9. Dabska tumor developing within a preexisting vascular malformation.

    Malignant endovascular papillary angioendothelioma, first described by Dabska in 1969, is a rare vascular tumor that primarily affects children and is characterized by papillary proliferations of endothelial cells into vessel lumens. We report a case of this rare neoplasm in a boy with angiomatosis who developed kasabach-merritt syndrome. The tumor evolved as an ulcerated lesion superficially within a previous vascular malformation on his buttock. A review of the literature is presented.
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ranking = 0.33333333333333
keywords = angiomatosis
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