Diffuse Dermal Angiomatosis Associated With Calciphylaxis

Abstract

Diffuse dermal angiomatosis (DDA) is a reactive proliferation of vascular channels within the dermis often associated with atherosclerosis. Based on our observation of a case of calciphylaxis (CP) with extensive DDA, we investigated a new possible association and incidence of DDA in patients with CP. These 2 rare conditions had not been reported previously in the same patient. In a retrospective review of skin biopsies taken between 1988 and 2006, 11 patients with histologically proven CP were identified and the medical records were reviewed. Two cases were excluded due to inadequate specimens for a thorough histologic evaluation. Nine patients with large necrotic plaques/ulcers were included in the study. Associated diseases were end-stage renal insufficiency (n = 7), parathyroidectomy for hyperparathyroidism (n = 3), thromboembolic events (n = 3), hypertension (n = 3), and diabetes mellitus (n = 2). Histologically, all cases had some degree of diffuse dermal proliferation of vascular channels with interstitial expression of CD31, as well as subcutaneous fat necrosis and calcification with medial vascular calcification. The extent of DDA did not correlate with the gravity or severity of disease. Based on our observation, DDA is a common histological finding encountered in the dermis adjacent to necrotizing ulcers in patients with CP.

Diffuse dermal angiomatosis associated with calciphylaxis in a patient with end‐stage renal disease

“…In a retrospective review of nine patients with biopsyproven calciphylaxis, all cases had histopathologic evidence of diffuse proliferation of endothelial cells between collagen bundles in the papillary and upper reticular dermis, consistent with DDA. 4 Similar to our patient, most of these patients had ESRD on dialysis, and presented with painful necrotic plaques involving the lower extremities (Table 1).…”

“…A hypercoagulable state may predispose to this vascular thrombosis, as protein C deficiency and antiphospholipid syndrome have been reported in patients with calciphylaxis. 4,15 The histopathologic findings of calciphylaxis, including medial calcification, subintimal fibroplasia and thrombosis of arteries and arterioles in the dermis and necrosis of subcutis, dermis and epidermis, support this proposed pathogenesis. 13 An ischemic event appears to be a critical step in the development of calciphylaxis and DDA with different responses.…”

“…The lower-grade ischemia that leads to DDA may precede the frank ischemia seen in calciphylaxis, and DDA may represent an early pathologic sign that heralds the possibility that calciphylaxis may subsequently ensue. 4 Alternatively, the vascular occlusion of larger dermal vessels in calciphylaxis may drive an increase in levels of VEGF as a compensatory mechanism, suggesting that DDA may occur as a secondary response to calciphylaxis. Early detection of DDA lesions may help clinicians identify patients at risk for developing calciphylaxis and mitigate their risk by effectively managing comorbid conditions, avoiding precipitating medications or controlling of serum calcium and phosphorus levels.…”

“…4 We present a case of a 72-yearold male with ESRD on peritoneal dialysis who was found to have concomitant calciphylaxis and DDA.…”

Diffuse dermal angiomatosis associated with severe atherosclerosis: two cases and review of the literature

“…In both patients, no evidence of upstream arterial stenosis was found, suggesting that occlusion of small arterioles by atherosclerosis may have triggered the disease in our cases. Another study found that DDA was a common finding in the dermis of patients with calciphylaxis, suggesting the role of arteriole obliteration in the physiopathology of DDA …”