New algorithm (KAWAKAMI algorithm) to diagnose primary cutaneous vasculitis
- 3 February 2010
- journal article
- review article
- Published by Wiley in The Journal of Dermatology
- Vol. 37 (2), 113-124
- https://doi.org/10.1111/j.1346-8138.2009.00761.x
Abstract
Palpable purpura tends to indicate involvement of small vessel vasculitis in the upper dermis. Livedo racemosa, nodular lesion and skin ulceration are indicative of involvement of small to medium-sized vessel vasculitis in the lower dermis to subcutaneous fat. We set out to establish a new algorithm (KAWAKAMI algorithm) for primary cutaneous vasculitis based on the Chapel Hill Consensus Conference classification and our research results, and apply to the diagnosis. The first step is to measure serum antineutrophil cytoplasmic antibodies (ANCA) levels. If myeloperoxidase-ANCA is positive, Churg-Strauss syndrome or microscopic polyangiitis can be suspected, and if the patient is positive for proteinase 3-ANCA, Wegener's granulomatosis is most likely. Next, if cryoglobulin is positive, cryoglobulinemic vasculitis should be suspected. Third, if direct immunofluorescence of the skin biopsy specimen reveals immunoglobulin A deposition within the affected vessels, Henoch-Schönlein purpura is indicated. Finally, the presence of anti-phosphatidylserine-prothrombin complex antibodies and/or lupus anticoagulant and histopathological necrotizing vasculitis in the upper to middle dermis (leukocytoclastic vasculitis) indicates cutaneous leukocytoclastic angiitis, whereas if necrotizing vasculitis exists in the lower dermis and/or is associated with the subcutaneous fat, cutaneous polyarteritis nodosa is indicated. The KAWAKAMI algorithm may allow us to refine our earlier diagnostic strategies and allow for efficacious treatment of primary cutaneous vasculitis. In cutaneous polyarteritis nodosa, warfarin or clopidogrel therapies should be administrated, and in cases that have associated active inflammatory lesions, corticosteroids or mizoribine (mycophenolate mofetil) therapy should be added. We further propose prophylactic treatment of renal complications in patients with Henoch-Schönlein purpura.Keywords
This publication has 57 references indexed in Scilit:
- High titer of anti–phosphatidylserine‐prothrombin complex antibodies in patients with cutaneous polyarteritis nodosaArthritis Care & Research, 2007
- Effects of Corticosteroid on Henoch-Schönlein Purpura: A Systematic ReviewPEDIATRICS, 2007
- Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studiesAnnals Of The Rheumatic Diseases, 2006
- Elevated serum IgA anticardiolipin antibody levels in adult Henoch-Schönlein purpuraBritish Journal of Dermatology, 2006
- Argatroban: UpdateAmerican Heart Journal, 2006
- Livedo reticularis: An updateJournal of the American Academy of Dermatology, 2005
- Mizoribine oral pulse therapy for patients with disease flare of lupus nephritisClinical Nephrology, 2003
- Functional Interaction of the Immunosuppressant Mizoribine with the 14-3-3 ProteinBiochemical and Biophysical Research Communications, 2000
- Antineutrophil Cytoplasmic Autoantibodies Interact with Primary Granule Constituents on the Surface of Apoptotic Neutrophils in the Absence of Neutrophil PrimingThe Journal of Experimental Medicine, 1996
- New Immunosuppressive Drugs in Organ TransplantationThe Journal of Clinical Pharmacology, 1996