Cutaneous Vasculitis Update: Neutrophilic Muscular Vessel and Eosinophilic, Granulomatous, and Lymphocytic Vasculitis Syndromes

Abstract

Most 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.

New algorithm (KAWAKAMI algorithm) to diagnose primary cutaneous vasculitis

“…7,8 For most physicians, cutaneous vasculitis evokes a crop of lower-extremity palpable purpura that correlates histologically with small vessel necrotizing vasculitis. 9 For dermatologists, it would be preferable to devise a classification system dedicated to those vasculitides that present cutaneously. 8 Given the current lack of knowledge regarding the pathogenesis, vessel size has been used to diagnose this condition because size can be ascertained relatively easily by clinical and histopathological examination.…”

Is lymphocytic vasculitis predictive pattern for lupus erythematosus?

“…LV paterni; LE, Jessner'in lenfositik infiltrasyonu yanısıra toksik eritem, kollajen vasküler hastalıklar, gebeliğin polimorfik erüpsiyonu, gebeliğin prurigosu, eritema annulare sentrifugum, pitriyazis rosea, pigmente purpurik dermatoz, piyoderma gangrenosum, polimorfik ışık erüpsiyonu, TRAPS, lösemik vaskülit, toksik şok sendromu, viral ve riketsiyal infeksiyonlar, perniosis, "ice pack" dermatozu, PLEVA gibi likenoid hastalıklar, ilaç erüpsiyonları, artropod ısırığı, Behçet hastalığı, Degos hastalığı, Sneddon sendromu, Kawasaki hastalığı ve süperfisyel tromboflebit gibi farklı birçok hastalıkta tanımlanmıştır (1,10,11).…”

Systemic vasculitis with cutaneous involvement, in the Department of Dermatology, Faculty of Medical Sciences, National University of Asuncion

“…En este trabajo de investigación, que abarca un período de 12 años en la Cátedra de Dermatología FCM -UNA, se ha encontrado que el 0,11% del total de consultas corresponden a vasculitis en general; y que dentro de este grupo, las vasculitis sistémicas con compromiso cutáneo representan el 8,66% de las consultas; lo que concuerda con la literatura y las publicaciones internacionales (3,(8)(9)(10) de que es una patología de baja incidencia y prevalencia. En el estudio realizado por Ochoa D., y col. (10), sobre la epidemiología de las vasculitis primarias en Colombia, se refiere una incidencia promedio de 0,3 a 20 casos por millón de habitantes.…”