Cutaneous lymphocytic thrombophilic (macular) arteritis |
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| Affiliation: | 1. Departments of Dermatology and Dermatopathology, Saint-Pierre, Brugmann and HUDERF Hospitals, Université Libre de Bruxelles (ULB), Brussels, Belgium;2. CTA Pathology and Departments of Dermatology and Pathology, Oregon Health and Science University, Portland, Oregon, USA;1. Chicago Medical School at Rosalind Franklin University, North Chicago, Illinois, USA;2. Western Michigan University Homer Stryker MD School of Medicine, Kalamazoo, Michigan, USA;3. Department of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, Pennsylvania, USA;1. Virginia Tech Carilion School of Medicine, Roanoke, Virginia, USA;2. Department of Dermatology, Tulane University School of Medicine, New Orleans, Louisiana, USA;3. Department of Dermatology, University of Florida College of Medicine, Gainesville, Florida, USA |
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| Abstract: | Macular arteritis (MA) has a striking discordance between the clinical presentation of hyperpigmented macules and the histopathologic findings of a lymphocytic arteritis with intraluminal hyalinized fibrin ring and thrombosis. It has been proposed that MA represents the chronic, indolent, lymphocytic form of the neutrophil-predominant cutaneous polyarteritis nodosa. MA usually affects middle-aged women asymptomatically on the legs. There is also a slightly more severe variant with more infiltrated plaques and livedo racemosa, termed lymphocytic thrombophilic arteritis. MA and lymphocytic thrombophilic arteritis have similar histologic features, both with a largely intact vascular elastic lamina, despite the abundant fibrin and endarteritis obliterans. There is no evidence for progression from MA to lymphocytic thrombophilic arteritis to cutaneous polyarteritis nodosa, and aggressive therapy should be avoided in MA, given the indolent, benign disease course. |
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