Diffuse dermal angiomatosis: A variant of reactive angioendotheliomatosis associated with atherosclerosis
Authors: Arash Kimyai-Asadi, Hossein C. Nousari, Nooshin Ketabchi, Jean M. Henneberry, Constantino Costarangos
Journal: Journal of the American Academy of Dermatology (1999)

Reactive angioendotheliomatosis (RAE) is a rare self-limited cutaneous disease characterized by intravascular endothelial cell proliferation.1Wick MR Rocamora A Reactive and malignant "angioendotheliomatosis": a discriminant clinicopathological study.J Cutan Pathol. 1988; 15: 260-271Crossref PubMed Scopus (81) Google Scholar A clinicopathologic variant of RAE associated with atherosclerosis has recently been described and termed diffuse dermal angiomatosis (DDA).2Krell JM Sanchez RL Solomon AR Diffuse dermal angiomatosis: a variant of reactive cutaneous angioendotheliomatosis.J Cutan Pathol. 1994; 21: 363-370Crossref PubMed Scopus (93) Google Scholar We present the third reported case of DDA. A 57-year-old white woman with a history of hypertension, smoking, debilitating bilateral leg claudication, and chronic renal insufficiency presented with a 3-week history of painful inner thigh lesions. Her medications included warfarin, amlodipine, lisinopril, hydrochlorothiazide, triamterene, and oxycodone. Physical examination revealed a 12 cm blanchable, reticulated, erythematous plaque with a 3 cm purpuric and necrotic area in the left upper medial thigh (Fig 1), and tender reticulated erythematous plaques on the upper medial right thigh and left medial ankle. The patient's blood urea nitrogen, creatinine, and cholesterol values were 31, 1.7, and 274 mg/dL, respectively. Values of electrolytes, liver function tests, complete blood cell count, calcium, phosphate, urinalysis, prothrombin time, activated partial thromboplastin time, erythrocyte sedimentation rate, anticardiolipin antibodies, parathyroid hormone, antineutrophil cytoplasmic antibodies, antinuclear antibodies, rheumatoid factor, anti-Ro, anti-La, anti-Sm, DNA, and ribonucleoprotein antibodies, cryoglobulins, cryofibrinogen, rapid plasma reagin, Russell viper venom time, factor V Leiden, and proteins C and S were normal. Computed tomography, magnetic resonance imaging, and angiography revealed an atrophic left kidney as well as complete occlusion of the left renal artery and the infrarenal aorta with collateral flow to the lower extremities. An incisional biopsy specimen of the left thigh lesion revealed striking proliferation of small vessels in the papillary and mid dermis with a moderate interstitial mononuclear cell infiltrate and no evidence of cholesterol emboli or vasculitis (Fig 2, A ). Immunoperoxidase studies using CD34 and CD31 markers highlighted the vessels, confirming the vascular nature of the process (Fig 2, B ). CD68 and common leukocyte antigen staining characterized the infiltrate as a mixture of histiocytes and lymphocytes. The patient subsequently underwent aortobifemoral bypass surgery, which resulted in complete clearing of her cutaneous lesions within 6 weeks. Angioendotheliomatosis is a rare disease characterized by erythematous purpuric plaques that occasionally ulcerate.3Requena L Sangueza OP Cutaneous vascular proliferations. Part III. Malignant neoplasms, other cutaneous neoplasms with significant vascular component, and disorders erroneously considered vascular neoplasms.J Am Acad Dermatol. 1998; 38: 143-175Abstract Full Text Full Text PDF PubMed Scopus (179) Google Scholar Recent studies indicate that, despite similar cutaneous findings, there are two forms of angioendotheliomatosis: malignant and reactive. Malignant angioendotheliomatosis is a fatal multisystem angiotropic lymphoma characterized histopathologically by dilated vessels filled with atypical mononuclear cells.3Requena L Sangueza OP Cutaneous vascular proliferations. Part III. Malignant neoplasms, other cutaneous neoplasms with significant vascular component, and disorders erroneously considered vascular neoplasms.J Am Acad Dermatol. 1998; 38: 143-175Abstract Full Text Full Text PDF PubMed Scopus (179) Google Scholar In contrast, RAE is a benign, self-limited disease associated with infections and cryoglobulinemia.4Lazova R Slater C Glynis S Reactive angioendotheliomatosis: case report and review of the literature.Am J Dermatopathol. 1996; 18: 63-69Crossref PubMed Scopus (56) Google Scholar, 5LeBoit PE Solomon AR Santa Cruz DJ Wick MR Angiomatosis with luminal cryoprotein deposition.J Am Acad Dermatol. 1992; 27: 963-973Abstract Full Text PDF Scopus (57) Google Scholar Histopathologically, there are dilated dermal vessels partially occluded by benign hyperplastic endothelial cells, forming prominent glomeruloid structures.1Wick MR Rocamora A Reactive and malignant "angioendotheliomatosis": a discriminant clinicopathological study.J Cutan Pathol. 1988; 15: 260-271Crossref PubMed Scopus (81) Google Scholar DDA, a variant of RAE associated with severe atherosclerotic disease, has been previously reported in 2 patients.2Krell JM Sanchez RL Solomon AR Diffuse dermal angiomatosis: a variant of reactive cutaneous angioendotheliomatosis.J Cutan Pathol. 1994; 21: 363-370Crossref PubMed Scopus (93) Google Scholar The cutaneous lesions of DDA are clinically indistinguishable from those of RAE, but occur in areas of vascular insufficiency and clear completely with revascularization.2Krell JM Sanchez RL Solomon AR Diffuse dermal angiomatosis: a variant of reactive cutaneous angioendotheliomatosis.J Cutan Pathol. 1994; 21: 363-370Crossref PubMed Scopus (93) Google Scholar Histopathologically, the dilated glomeruloid vessels characteristic of RAE are absent in DDA. Rather, hyperplastic endothelial cells diffusely infiltrate the reticular dermis forming small lumens.2Krell JM Sanchez RL Solomon AR Diffuse dermal angiomatosis: a variant of reactive cutaneous angioendotheliomatosis.J Cutan Pathol. 1994; 21: 363-370Crossref PubMed Scopus (93) Google Scholar, 3Requena L Sangueza OP Cutaneous vascular proliferations. Part III. Malignant neoplasms, other cutaneous neoplasms with significant vascular component, and disorders erroneously considered vascular neoplasms.J Am Acad Dermatol. 1998; 38: 143-175Abstract Full Text Full Text PDF PubMed Scopus (179) Google Scholar This patient had cutaneous lesions characteristic of angioendotheliomatosis in areas of vascular insufficiency. Diffuse rather than lobular dermal small vessel proliferation, absence of dilated vasculature, and complete clearing with revascularization were consistent with a diagnosis of DDA.2Krell JM Sanchez RL Solomon AR Diffuse dermal angiomatosis: a variant of reactive cutaneous angioendotheliomatosis.J Cutan Pathol. 1994; 21: 363-370Crossref PubMed Scopus (93) Google Scholar, 3Requena L Sangueza OP Cutaneous vascular proliferations. Part III. Malignant neoplasms, other cutaneous neoplasms with significant vascular component, and disorders erroneously considered vascular neoplasms.J Am Acad Dermatol. 1998; 38: 143-175Abstract Full Text Full Text PDF PubMed Scopus (179) Google Scholar This case supports previous evidence that DDA is a clinicopathologic variant of RAE associated with atherosclerosis.