Intravascular Cutaneous Disorders. A Clinicopathologic Review

Abstract

Intravascular proliferations of the skin are clinically heterogeneous and may present with a wide range of clinical features, including violaceous papules, nodules, plaques, or other unspecific cutaneous lesions. Histopathologically, these conditions are characterized by proliferation of different cell types within the lumina of dermal vessels and endothelial cell hyperplasia. Immunohistochemistry is the best tool to identify the nature of the intravascular proliferating cells and the type of involved vessel. In this review, we analyzed the clinicopathologic and immunohistochemical characteristics of intravascular large cell lymphoma, T-cell and natural killer-cell intravascular large cell lymphoma, intralymphatic variant of CD30+ cutaneous lymphoproliferative disorders, benign atypical intralymphatic CD30+ T-cell proliferation, reactive angioendotheliomatosis, intralymphatic histiocytosis, papillary intralymphatic angioendothelioma or Dabska tumor, glomeruloid hemangioma, papillary hemangioma, intravascular papillary endothelial hyperplasia or Masson phenomenon, and the intralymphatic involvement of Merkel cell carcinoma, cutaneous metastases, and cutaneous angiosarcoma.

Sudden Development of Indurated Subcutaneous Nodules in a Patient With a Recent Melanoma Surgical Procedure

“…Each papillary frond is covered by plump endothelial cells . These tumors are commonly D2-40 negative and CD31, CD34, alpha smooth muscle actin, and factor VIII–related antigen positive in cells lining the papillae of Masson tumor …”

Indurated Plaques With Telangiectasias on the Lower Limbs

“…Generally, cell morphology, immunohistochemistry, and the type of vessels affected are key to differentiate IVLBCL from other conditions. In intralymphatic histiocytosis, a reactive phenomenon secondary to chronic cutaneous infections, connective tissue disorders, or surgical scars, clusters of histiocytoid cells (expressing CD68 and CD163) are floating within the lumina of dilated vessels . The structures affected are lymphatic vessels (positive for CD31, CD34, and podoplanin).…”

“…The structures affected are lymphatic vessels (positive for CD31, CD34, and podoplanin). Reactive angioendotheliomatosis is a benign intravascular proliferation of endothelial cells of unknown etiology that has been described in association with systemic infections, microvascular occlusive disorders, and rheumatoid arthritis. The histopathologic spectrum of reactive angioendotheliomatosis is quite broad.…”

“…It has been speculated that it could represent a population of activated CD30 + T cells that migrate to the lymph nodes in response to an unknown antigen. Intralymphatic proliferation of T-cell lymphoid blasts are characterized by the presence of large, blastoid T lymphocytes within lymphatic vessels . These lymphocytes often express CD30 and lack the monoclonal rearrangement of the T-cell receptor genes.…”

Characterization of intravascular papillary endothelial hyperplasia: a multicentre cohort

“…IPEH is classically categorized into three types: (i) a primary/pure type arising in dilated endovascular spaces; (ii) a secondary/mixed type developing from pre‐existing vascular abnormalities such as varices, haemangiomas and/or arteriovenous malformations; and (iii) a third/extravascular type arising from haematomas 1,2 . Regardless of anatomical site, IPEH is a reactive process involving endothelial cell proliferation within an organizing thrombus 3 . Therefore, although the prognosis of IPEH is excellent, it must be differentiated from malignant tumours such as angiosarcoma, which may require intensive treatments 2 .…”