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.
Keywords: vasculitispolyarteritis nodosamedium vessel vasculitis
References
- De Virgilio A, Greco A, Conte M, et al. Polyarteritis nodosa: A contemporary overview. Autoimmun Rev. 2016; 15(6): 564–570.
- Zhou Q, Yang D, Ombrello AK, et al. Early-onset stroke and vasculopathy associated with mutations in ADA2. N Engl J Med. 2014; 370(10): 911–920.
- Chasset F, Francès C. Cutaneous Manifestations of Medium- and Large-Vessel Vasculitis. Clin Rev Allergy Immunol. 2017; 53(3): 452–468.
- Soowamber M, Weizman AV, Pagnoux C. Gastrointestinal aspects of vasculitides. Nat Rev Gastroenterol Hepatol. 2017; 14(3): 185–194.
- Kluger N, Pagnoux C, Guillevin L, et al. French Vasculitis Study Group. Comparison of cutaneous manifestations in systemic polyarteritis nodosa and microscopic polyangiitis. Br J Dermatol. 2008; 159(3): 615–620.
- Lightfoot RW, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. 1990; 33(8): 1088–1093.
- Yates M, Watts RA, Bajema IM, et al. European Vasculitis Study Group. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009; 68(3): 310–317.
- Criado PR, Marques GF, Morita TC, et al. Epidemiological, clinical and laboratory profiles of cutaneous polyarteritis nodosa patients: Report of 22 cases and literature review. Autoimmun Rev. 2016; 15(6): 558–563.
- Nakamura T, Kanazawa N, Ikeda T, et al. Cutaneous polyarteritis nodosa: revisiting its definition and diagnostic criteria. Arch Dermatol Res. 2009; 301(1): 117–121.
- Daoud MS, Hutton KP, Gibson LE. Cutaneous periarteritis nodosa: a clinicopathological study of 79 cases. Br J Dermatol. 1997; 136(5): 706–713.
- Bansal NK, Houghton KM. Cutaneous polyarteritis nodosa in childhood: a case report and review of the literature. Arthritis. 2010; 2010: 687547.
- Soufir N, Descamps V, Crickx B, et al. Hepatitis C virus infection in cutaneous polyarteritis nodosa: a retrospective study of 16 cases. Arch Dermatol. 1999; 135(8): 1001–1002.
- Imanishi H, Tsuruta D, Oshimo T, et al. Cutaneous polyarteritis nodosa induced by Mycobacterium tuberculosis. J Dermatol. 2012; 39(8): 738–739.
- Ambrosio MR, Rocca BJ, Ginori A, et al. Renal infarction due to polyarteritis nodosa in a patient with angioimmunoblastic T-cell lymphoma: a case report and a brief review of the literature. Diagn Pathol. 2012; 7: 50.
- Culver B, Itkin A, Pischel K. Case report and review of minocycline-induced cutaneous polyarteritis nodosa. Arthritis Rheum. 2005; 53(3): 468–470.
- Morgan AJ, Schwartz RA. Cutaneous polyarteritis nodosa: a comprehensive review. Int J Dermatol. 2010; 49(7): 750–756.
- Criado PR, Marques GF, Morita TC, et al. Epidemiological, clinical and laboratory profiles of cutaneous polyarteritis nodosa patients: Report of 22 cases and literature review. Autoimmun Rev. 2016; 15(6): 558–563.
- de Menthon M, Mahr A. Treating polyarteritis nodosa: current state of the art. Clin Exp Rheumatol. 2011; 29(1 Suppl 64): S110–S116.
- Krishnan S, Bhakuni DS, Kartik S. Rituximab in refractory cutaneous polyarteritis. Int J Rheum Dis. 2012; 15(5): e127.
- Kluger N, Guillot B, Bessis D. Ulcerative cutaneous polyarteritis nodosa treated with mycophenolate mofetil and pentoxifylline. J Dermatolog Treat. 2011; 22(3): 175–177.
- Buffiere-Morgado A, Battistella M, Vignon-Pennamen MD, et al. Relationship between cutaneous polyarteritis nodosa (cPAN) and macular lymphocytic arteritis (MLA): Blinded histologic assessment of 35 cPAN cases. J Am Acad Dermatol. 2015; 73(6): 1013–1020.
![](https://journals.viamedica.pl/plugins/generic/popups/images/icons/close.png)