Urticarial vasculitis

Abstract

Urticarial vasculitis can present in a variety of ways, ranging from a primarily cutaneous disease consisting of chronic urticaria to a lupus-like disease with severe cardiopulmonary disease. Low complement levels and positive anti-C1q antibodies are markers of more severe disease. Care must be taken to look for an underlying condition. The mainstay of therapy is treatment of any underlying condition. Therapies most often employed include corticosteroids, antihistamine, and dapsone, but many others have been utilized.

Urticarial Syndromes

“…Leukocytoclastic vasculitis of the small dermal vessels characterized by a neutrophilic perivascular iniltrate, the typical indings for UV, is observed in fully developed lesions. Additionally, neutrophil fragmentation, nuclear dust, erythrocyte extravasation, and ibrin deposition in and around the vessels are observed [50,52].…”

“…Urticarial lesions continue longer than 24-36 h. Central clearing of lesions is seen in time and they are accompanied by palpable purpura. Necrosis and ulceration are less common skin indings [50]. The lesions regress with a residual hyperpigmentation [47,51].…”

“…However, an association with various drugs, sun, cold, connective tissue diseases, infections, and various malignancies (paraneoplastic) has been identiied [50,51,53]. Among connective tissue diseases, it is most commonly seen with systemic lupus erythematosus (SLE) [53].…”

“…The most common systemic manifestations are in the joints, kidneys, and lungs [52,54]. Gastrointestinal and neurologic involvement can also be seen [50,52]. Antinuclear antibody (ANA) positivity has also been reported in up to 78% of HUV patients [52,54].…”

“…Steroid-sparing agents are used in the treatment to avoid the side efects of long-term corticosteroids. Dapsone, colchicine, hydroxychloroquine, mycophenolate mofetil, interferon-alpha, cyclosporine A, azathioprine, cyclophosphamide, rituximab, intravenous immunoglobulins, anakinra, and plasmapheresis are treatment agents that can be used alone or in combination with corticosteroids [50][51][52].…”