Cryoglobulinaemia and angiomatosis*

Harper, J.I.; Gray, William D.; Wilson‐Jones, E.

doi:10.1111/j.1365-2133.1983.tb04620.x

Cited by 13 publications

(2 citation statements)

References 10 publications

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“…1,20 Histopathologically, these disorders show an intravascular or extravascular lobular or diffuse hyperplasia of endothelial cells, There have been nine previously reported cases of reactive angiomatosis secondary to cryoproteins, three of which were found to be associated with an underlying hematologic malignancy (Table 2). [28][29][30][31][32][33][34][35] In the first such case associated with underlying malignancy, similar to our case, workup of cryoglobulinemia revealed lowgrade lymphoplasmacytic lymphoma. 28 In another case, the initial presentation of angiomatosis with cryoproteins preceded a diagnosis of B-chronic lymphocytic leukemia by 3 years.…”

Section: Intraluminal Glomeruli-like Proliferation Of Capillariessupporting

confidence: 74%

Cutaneous reactive angiomatosis associated with intravascular cryoprotein deposition as the presenting finding in a patient with underlying lymphoplasmacytic lymphoma: A case report and review of the literature

et al. 2021

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Cutaneous reactive angiomatosis, a group of disorders defined by benign vascular proliferation, is associated with a number of systemic processes, including intravascular occlusion by cryoproteins. We report a case of a 64-year-old female patient who presented with a 1-year history of nontender petechiae of the bilateral arms and lower legs. Dermoscopic evaluation showed increased vascularity with a globular pattern. Over a period of months, her findings progressed to erythematous to violaceous plaques with admixed hypopigmented stellate scarring of the bilateral lower extremities, forearms, and lateral neck. Biopsy showed increased thin-walled, small dermal blood vessels with focal inter-anastamosis. Some vessels were occluded by eosinophilic globules suspicious for cryoprotein. Subsequent laboratory studies confirmed a diagnosis of type 1 cryoglobulinemia, prompting a bone marrow biopsy that revealed lymphoplasmacytic lymphoma. Herein, we report the fourth case of angiomatosis secondary to intravascular cryoproteins as the initial presentation of an underlying hematologic malignancy. We also present a review of the literature and emphasize the need for thorough initial workup and close and prolonged clinical monitoring for underlying systemic disease in these patients.

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Section: Intraluminal Glomeruli-like Proliferation Of Capillariessupporting

confidence: 74%

Cutaneous reactive angiomatosis associated with intravascular cryoprotein deposition as the presenting finding in a patient with underlying lymphoplasmacytic lymphoma: A case report and review of the literature

et al. 2021

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“…In addition, multiple biopsies showed intravascular organizing fibrin thrombi associated with proliferation of endothelial cells raising the differential diagnosis of reactive angioendotheliomatosis (RAE). This rare disorder and related cutaneous reactive angiomatoses show intravascular proliferation within dermal capillaries with variable but mostly minimal inflammation and common luminal obstruction by fibrin thrombi or abnormal proteins including cryoglobulins, cryofibrinogen, cold agglutinins and immunoglobulins. Sometimes glomeruloid structures are seen .…”

Section: Discussionmentioning

confidence: 99%

Cutaneous collagenous vasculopathy associated with intravascular occlusive fibrin thrombi

2014

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Cutaneous collagenous vasculopathy (CCV) is a rare cutaneous microangiopathy that clinically resembles generalized essential telangiectasia with only 12 cases reported to date. The perivascular fibrosis is thought to be due to production of abnormal collagen by veil cells in the outer vessel walls as a result of unknown factors. This report is of an 84-year-old male with progressive telangiectasia. Biopsies showed characteristic features of CCV. In addition, there were multiple intravascular fibrin thrombi, some organizing and associated with endothelial cell hyperplasia with recanalization reminiscent of glomeruloid bodies and simulating reactive angioendotheliomatosis (RAE). Histochemically and ultrastructurally fibrin was noted within the vessel walls integrating into the fibrous tissue around the vessels; however, the patient had no evidence of coagulation disorder, cryoglobulinemia or cold agglutinemia. Immunofluorescence showed fibrinogen within the vessel walls but no immunoglobulins or C3. As well, there were minimal inflammatory cells. This suggests pauci-inflammatory injury to the endothelial cells by unknown angiogenic factors causing local intravascular fibrin thrombi with fibrin leaking and incorporating into the vessel walls, eventually leading to reparative perivascular fibrosis. This case suggests that some cases of CCV are related to a primary local intravascular occlusive thrombotic microangiopathy. However, the primary triggering factor causing the endothelial cell damage has yet to be elucidated.

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