Lymphocytic Thrombophilic Arteritis
Open Access
- 1 September 2008
- journal article
- observation
- Published by American Medical Association (AMA) in Archives of Dermatology
- Vol. 144 (9), 1175-1182
- https://doi.org/10.1001/archderm.144.9.1175
Abstract
Background We encountered a distinct arteriolar histopathologic finding of lymphocytic vasculitis associated with a hyalinized fibrin ring in vessel lumina. Identical histologic findings have previously been described as macular arteritis. Observations We describe 5 women (mean age, 25 years; age range, 20-34 years) with persistent, slowly progressive, patchy and reticular hyperpigmentation associated with livedo racemosa affecting predominantly the lower limbs. In the biopsy samples, infiltration of muscular vessel wall by inflammatory cells, affecting small arteries of the dermosubcutaneous junction or superficial subcutis, was present. Of the infiltrate, 90% or more consisted of mononuclear cells, mainly lymphocytes with an admixture of histiocytes. Neutrophils and eosinophils were absent or scant. Inflammation was confined to the vicinity of the vessel and the immediate surrounding panniculus. A concentric fibrin ring involving the entire periphery of the lumina of affected vessels was present in all the patients. Laboratory investigation results revealed that 4 patients had antiphospholipid antibodies in their serum. One of these patients had a heterozygous mutation of the factor V Leiden gene. Conclusion We term this arteritislymphocytic thrombophilic arteritisto reflect the histologic features that combine lymphocytic vascular inflammation with changes representing a thrombophilic endovasculitis.Keywords
This publication has 20 references indexed in Scilit:
- Antiphospholipid antibodies and the antiphospholipid syndrome: an update on treatment and pathogenic mechanismsCurrent Opinion in HIV and AIDS, 2006
- International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS)Journal of Thrombosis and Haemostasis, 2006
- Cutaneous Vasculitis Update: Diagnostic Criteria, Classification, Epidemiology, Etiology, Pathogenesis, Evaluation and PrognosisThe American Journal of Dermatopathology, 2005
- Livedo (livedoid) vasculitis and the factor V Leiden mutation: Additional evidence for abnormal coagulationJournal of the American Academy of Dermatology, 2002
- Defining lymphocytic vasculitis.Australasian Journal of Dermatology, 2000
- Nomenclature of Systemic Vasculitides: Proposal of an International Consensus ConferenceRadiology, 1995
- The American college of rheumatology 1990 criteria for the classification of polyarteritis nodosaArthritis & Rheumatism, 1990
- The American College of Rheumatology 1990 criteria for the classification of vasculitis: IntroductionArthritis & Rheumatism, 1990
- Histopathologic and immunofluorescence study of skin lesions associated with circulating lupus anticoagulantJournal of the American Academy of Dermatology, 1988
- Livedo vasculitis. Segmental hyalinizing vasculitis of the dermisArchives of Dermatology, 1967