Diffuse dermal angiomatosis of the breast: an emerging entity in the setting of cutaneous reactive angiomatoses

New and emerging types of cutaneous vascular (capillary) proliferations have been described or better categorized in the last few years. They include reactive angioendotheliomatosis, acroangiodermatitis (pseudo-Kaposi sarcoma), diffuse dermal angiomatosis, intravascular histiocytosis, glomeruloid angioendotheliomatosis, and angiopericytomatosis (angiomatosis with cryoproteins). Clinically, they are characterized by multiple, red violaceous, and purpuric patches and plaques, sometimes evolving toward necrosis and ulceration with a wide distribution but a propensity to involve the extremities. Histologically, they are characterized by different patterns of intravascular or extravascular lobular or diffuse hyperplasia of endothelial cells, pericytes, and sometimes histiocytes. Although these angioproliferations can histologically have a pseudoangiosarcomatous pattern, they are reactive in that they originate from the (sub)occlusion of vascular lumina by different localized or systemic disorders. The vascular proliferation stops after the inducing hypoxic stimulus has been withdrawn. Among them, diffuse dermal angiomatosis of the breast is a variant of diffuse dermal angiomatosis involving middle-aged women with macromastia, obesity, smoking, and vasculopathic disorders, considered a distinct disorder in the spectrum of cutaneous reactive angiomatoses. It presents with reticulated erythematous to purple patches with sometimes a tendency to ulcerate and bleeding, appearing on large, pendulous breasts. The pathogenesis is related to tissue hypoxemia resulting from subclinical torsion, compression, and increased venous hydrostatic pressure due to the macromastia, aggravated by the associated ischemic conditions such as hypertension and diabetes. There is no evidence-based therapy, but reduction mammoplasty is a viable treatment option. This should be evaluated in all patients who fail conservative therapy.