Polyarteritis Nodosa and Cutaneous Polyarteritis Nodosa

Courtney R. Herbert, MD, MPH, Glenn G. Russo, MD

Disclosures

Skinmed. 2003;2(5) 

In This Article

Abstract and Introduction

Cutaneous vasculitis can present with a variety of lesions and associated symptoms. It is important for the clinician to be able to recognize vasculitis lesions and know when to pursue further laboratory studies that will determine the extent of the disease process in a patient. This review compares and contrasts polyarteritis nodosa with its cutaneous counterpart. The diagnostic workup and management of these two entities as well as the overall approach to a patient with a possible vasculitic condition are emphasized.

Vasculitis is a clinicopathologic process characterized by inflammation and damage to blood vessels.[1] Clinical and laboratory abnormalities are nonspecific. Blood vessel injury results in increased permeability, edema, and purpura. Aneurysms, intimal proliferation, thrombosis, and hemorrhage result in stenosis and occlusion of the vessel wall.[2] Vasculitis can be primary or secondary. Primary vasculitides can be a primary manifestation of a disease and in fact the only disease a patient manifests. Mandell and Hoffman's discussion of primary systemic vasculitic disorders includes polyarteritis nodosa (PAN), Wegener's granulomatosis, Churg-Strauss syndrome, Kawasaki disease, Takayasu's arteritis, and temporal arteritis.[2] Secondary vasculitides include those caused by other diseases such as rheumatoid arthritis and systemic lupus erythematosus. With regard to the primary vasculitides, one needs to know the caliber of the blood vessels involved, pattern of organ involvement, if the process is granulomatous in nature, and finally, whether or not eosinophils are present.[2]

There are numerous primary vasculitides just as there are numerous targets (arteries, arterioles, venules, and capillaries). Large arteries, including the aorta and its major branches directed toward the extremities, and the head and neck are affected by giant cell or temporal arteritis and Takayasu's arteritis. Medium-sized vessels -- including the main visceral arteries such as the renal, hepatic, coronary, and mesenteric -- are affected by PAN and Kawasaki disease. Small arteries, such as the distal arteries connecting to arterioles and subcutaneous and dermal arteries, are affected by various disorders such as Wegener's granulomatosis, Churg-Strauss syndrome, Henoch-Schönlein purpura, cryoglobulinemia, and cutaneous leukocytoclastic vasculitis. Some small and large vessel vasculitides may involve medium-sized vessels, but large and medium-sized vessel vasculitides do not involve vessels smaller than arteries, and should not affect postcapillary venules in the dermis.[3]

This review focuses on PAN and cutaneous polyarteritis nodosa (CPAN) ( Table I ). PAN is defined as a systemic necrotizing vasculitis affecting medium-sized arteries in the kidney, liver, heart, and skin. CPAN is a vasculitis more limited in nature with its findings confined to the skin, musculoskeletal, and nervous system.[4]

The first case of PAN was described in 1866[3,5] and was originally named periarteritis nodosa due to the nodular protuberances along medium-sized vessels. In the early 1900s, it was discovered that there were inflammatory cells in all levels of the vessel and the name was subsequently changed to "polyarteritis nodosa." PAN was also the first vasculitis to be described.[5] The first case of CPAN was described in 1931.[5] Until that time, it was unclear that CPAN -- a benign and less severe form of vasculitis -- was a separate disorder. In 1980, investigators solidified the concept of CPAN as a distinct entity from systemic PAN.[6]

In 1990, the American College of Rheumatology[7] developed criteria for the classification of PAN in adults. The traditional format includes: weight loss >4 kg, livedo reticularis, testicular pain and tenderness, myalgias, mononeuropathy and or polyneuropathy, diastolic blood pressure >90 mm Hg, elevated blood urea nitrogen, elevated creatinine, positive hepatitis B surface antigen, arteriographic abnormalities, and granulocyte or mixed leukocyte infiltrates in the arterial wall on biopsy. This classification is associated with a specificity of 86% and sensitivity of 82% if three out of 10 criteria are present.[7] The pediatric criteria include: major criteria -- renal or musculoskeletal involvement; minor criteria -- cutaneous manifestations, gastrointestinal disease (abdominal pain to gastrointestinal bleeding); peripheral neuropathy, central nervous system disease including electroencephalographic abnormalities, hypertension/cardiovascular manifestations (pericarditis and arrhythmias), pulmonary symptoms (infiltrates, pleural effusions), constitutional symptoms, acute phase reactants, and presence of hepatitis B surface antigen. With the presence of five minor and one major criteria, this is highly suggestive of PAN in 97% of pediatric patients.[8] CPAN does not have any specific criteria; distinguishing characteristics are the lack of systemic involvement and its benign, yet relapsing, long-term course.[3] It was once hypothesized that cutaneous involvement varied inversely with the severity of visceral disease, yet this is not always the case. Some cases of CPAN do progress to PAN.[9] Some investigators have likened the relationship between CPAN and PAN to that of discoid lupus erythematosus and systemic lupus erythematosus.[10] A study of 20 CPAN patients undergoing long-term treatment and follow-up showed that two out of the 20 patients eventually progressed to PAN 18-19 years after their initial diagnosis of CPAN.[11] Another study showed a much higher incidence of seven out of nine CPAN patients eventually showing signs of systemic involvement over a period of 4 months to 14 years after the initial diagnosis of CPAN.[12]

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