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Cutaneous vasculitis update: neutrophilic muscular vessel and eosinophilic, granulomatous, and lymphocytic vasculitis syndromes.by: J. A. Carlson, K. R. Chen
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Notes for this articleAccording to some authorities,evidence of small vessel neutrophilic vasculitis is not required for diagnosis because urticarial vasculitis represents a continuum of histologic changes that range from sparse perivascular neutrophilic infiltrates, to intermediate lesions with denser perivascular neutrophilic infiltrates with nuclear debris (leukocytoclasia), extravasated red blood cells, but no fibrin deposits, to severe lesions with typical small vessel neutrophilic vasculitis. Because of the overlap between urticaria and cutaneous leukocytoclastic vasculitis, minimal histologic criteria for the diagnosis of UV have been proposed: nuclear debris or fibrin deposits with or without extravasated red blood cells.
For most cases of urticarial vasculitis, the cause is unknown; however, for a minority of patients, UV will represent one manifestation of a serious illness due to CTD such SLE and Sjogren’s syndrome (SS), compliment deficiencies such as C1q esterase deficiency, mixed cryoglobulinemia, and malignancies.
Urticarial vasculitis can be a manifestation of SLE: Hypocomplementemic UV patients tend to be female, have tissue neutrophilia (sparse interstitial as well as perivascular neutrophilic infiltrates) on skin biopsy, have a positive lupus band test (one or more granular basement membrane zone immunoreactants) on DIF and have co-existing CTD, most frequently systemic lupus erythematosus (SLE)
The hypocomplementemic UV syndrome describes UV patients who present with hypocomplementemia and SLE associated manifestations such as arthralgias or arthritis, glomerulonephritis, uveitis or episcleritis, recurrent abdominal pain, and/or obstructive lung disease. WHY DON'T YOU JUST SAY THIS IS LUPUS MANIFESTING CLINICALLY IN THE SKIN AS CHRONIC URTICARIAL ERUPTION.
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AbstractMost biopsies of cutaneous vasculitis will exhibit a small vessel neutrophilic vasculitis [leukocytoclastic vasculitis (LCV)] that is associated with immune complexes on direct immunofluorescence examination or, less commonly, antineutrophilic cytoplasmic antibodies (ANCA) by indirect immunofluorescence testing. Is in uncommon for skin biopsy to reveal solely a neutrophilic arteritis signifying the presence of cutaneous polyarteritis nodosa or, if accompanied by significant lobular panniculitis, nodular vasculitis/erythema induratum. In other cases, cutaneous vascular damage (fibrinoid necrosis, muscular vessel wall disruption, or endarteritis obliterans) will be mediated by a nonneutrophilic inflammatory infiltrate. Eosinophilic vasculitis can be a primary (idiopathic) process that overlaps with hypereosinophilic syndrome, or it can be a secondary vasculitis associated with connective tissue disease or parasite infestation. Authentic cutaneous granulomatous vasculitis (versus vasculitis with extravascular granulomas) can represent a cutaneous manifestation of giant cell arteritis, an eruption secondary to systemic disease such as Crohn's disease or sarcoidosis, or a localized disorder, often a post-herpes zoster (HZ) phenomenon. Lymphocytic vasculitis is a histologic reaction pattern that correlates with broad clinical differential diagnosis, which includes connective tissue disease - mostly systemic lupus erythematosus (SLE), endothelial infection by Rickettsia and viruses, idiopathic lichenoid dermatoses such as perniosis or ulcerative necrotic Mucha-Habermann disease, and angiocentric cutaneous T-cell lymphomas. Skin biopsy extending into the subcutis, identifying the dominant inflammatory cell and caliber of vessels affected, extravascular histologic clues such as presence of lichenoid dermatitis or panniculitis, and correlation with clinical data allows for accurate diagnosis of these uncommon vasculitic entities.
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