Cutaneous Vasculitis Update: Small Vessel Neutrophilic Vasculitis Syndromes

Abstract

A broad and diverse spectrum of vasculitic syndromes exists. These syndromes affect the skin with varying levels of associated systemic manifestations, running the gamut from a self-limited, localized, cutaneous phenomenon to rapidly progressive, multiorgan disease. The majority of cases of cutaneous vasculitis will show a neutrophilic small vessel vasculitis that can be either a primary (idiopathic) disorder (eg, cutaneous leukocytoclastic angiitis) or a secondary disorder that is associated with drugs, infection (eg, streptococcal infection, viral hepatitis), or underlying disease (eg, connective tissue disease, malignancy). Biopsy is the gold standard for the diagnosis of cutaneous vasculitis and also necessary for the detection of cutaneous vascular immune complexes by direct immunofluorescence. Based on the type of vessel disrupted by inflammation (small and/or muscular), the distribution of vasculitis in the dermis and subcutis, and predominate inflammatory cell-type mediating vessel wall damage, a list of relevant differential diagnoses can be generated. This histologic information coupled with extravascular findings such as tissue eosinophilia, tissue neutrophilia, and/or granulomas, plus pathophysiologic markers such as direct immunofluorescent examination for immune complexes and serologic evaluation for antineutrophil cytoplasmic antibodies allows for more accurate diagnosis of specific vasculitic entities. Herein, we review both primary and secondary vasculitic syndromes that affect the skin and show a small vessel neutrophilic mediated vasculitis.

The safety and tolerability of an HIV-1 DNA prime–protein boost vaccine (DP6-001) in healthy adult volunteers

“…These two cases of vasculitis are the first to be reported that were temporarily related to inoculation using HIV-1 Env in combination with the saponin adjuvant QS21 in humans. The etiology of LCV is not well understood but has been shown to be associated with immune complexes, autoantibodies such as antineutrophil cytoplasmic antibody (ANCA), and inflammatory mediators particularly those associated with endothelial adhesion molecules [23,24]. In the general population, hypersensitivity vasculitis occurs in 10-30 cases per million/ year and 14 cases of Henoch-Schonlein purpura per million/year have been reported [23] with up to one-half being idiopathic in nature.…”

A Case of Mistaken Identity: Henoch-Schonlein Purpura Masquerading as Urticaria

“…Proteolytic enzymes released by the neutrophils cause vessel wall damage and erythrocyte extravasation, resulting in palpable purpura [3]. Small, superficial vessel involvement can lead to urticarial lesions and purpura [7]. Deeper, dermal vessel involvement leads to hemorrhagic bullous or necrotic lesions [7].…”

S1 guidelines Diagnostics and treatment of ANCA-associated vasculitis

“…Die aus Hautbiopsien bei AAV am häufigsten nachweisbare leukozytoklastische Vaskulitis allein ist jedoch wenig spezifisch und kann eine Vielzahl von Ursachen haben [24,38].…”