Jm. Krell et al., DIFFUSE DERMAL ANGIOMATOSIS - A VARIANT OF REACTIVE CUTANEOUS ANGIOENDOTHELIOMATOSIS, Journal of cutaneous pathology, 21(4), 1994, pp. 363-370
Reactive angioendotheliomatosis (RA) is a rare, benign disease. Affect
ed patients present with self-limited, erythematous to violaceous plaq
ues. The clinical lesions are due to intravascular hyperplasia of cyto
logically banal endothelial cells in the dermis. We report 2 patients
who presented with ulcerated, violaceous plaques on the lower extremit
ies. Both had severe peripheral vascular atherosclerotic disease requi
ring bypass grafts. Unlike previously described cases of RA, our patie
nt's lesions were due to a diffuse proliferation of endothelial cells
in the reticular dermis with only minimal, focal intravascular prolife
ration of these cells. Positive immunostaining with antibodies to Fact
or VIII-related and CD34 antigens adds evidence that the proliferated
cells in the dermis were endothelial cells. (C) Munksgaard 1994.