Polyarteritis nodosa — clinical presentation, diagnosis and treatment

Abstract

Polyarteritis nodosa (PAN) belongs to the group of medium vessel vasculitis. Two subtypes of the disease are classically distinguished — systemic variant and cutaneous variant. Systemic PAN is a potentially life-threatening disease, while cutaneous subtype is usually characterized by mild course. The review aims to summarize the symptomatology, diagnostics and treatment of PAN and to draw attention to the dermatological manifestations of the disease. Skin changes develop in 28–60% of patients with systemic PAN. The most common manifestations include palpable purpura, livedo reticularis and inflammatory nodules, and less commonly - urticarial lesions, transient erythema, distal necrosis, superficial phlebitis and splinter haemorrhages. Skin lesions in cutaneous PAN are typically located on the lower extremities, and less frequently — on the upper extremities or trunk. Painful nodules, livedo reticularis and ulcerations are the most frequent manifestations of cutaneous PAN. Ulcerations usually heal leaving ivory-white stellate scars (atrophie blanche) surrounded by telangiectasias. The diagnosis of both systemic and cutaneous subtype of the disease is based on diagnostic criteria. Management of systemic PAN consists of induction of remission and maintenance therapy. The choice of therapeutic option should be based on the severity of the disease and comorbidities, especially hepatitis B virus infection. Cutaneous PAN is characterized by a benign course with frequent relapses. First-line treatment usually consists of non-steroidal anti-inflammatory drugs, colchicine and dapsone. Considering frequent development of skin lesions in the course of PAN, knowledge of the clinical presentation, diagnosis and treatment of the condition is an important part of the everyday dermatological practice.