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Cutaneous polyarteritis nodosa

Author
Robert G Micheletti, MD
Section Editors
Jeffrey Callen, MD, FACP, FAAD
Peter A Merkel, MD, MPH
Deputy Editors
Abena O Ofori, MD
Monica Ramirez Curtis, MD, MPH

INTRODUCTION

Cutaneous polyarteritis nodosa (cPAN) is a type of vasculitis affecting medium-sized vessels in the skin, a distinct subset of polyarteritis nodosa (PAN) first described in 1931 [1]. Whereas classic systemic PAN commonly affects the kidneys, joints, muscles, nerves, and gastrointestinal tract, as well as the skin, vasculitis in cPAN is skin-limited. (See "Clinical manifestations and diagnosis of polyarteritis nodosa in adults".)

Sometimes called "benign cutaneous PAN," cPAN can nevertheless result in significant morbidity due to painful skin nodules, digital infarcts, ulcerations, and other complications (picture 1A-E). Mild systemic symptoms, which must be distinguished from systemic vasculitis, may also develop.

The cutaneous manifestations of cPAN and systemic PAN are similar; therefore, all patients with cPAN should be evaluated for systemic involvement at the time of diagnosis. In addition, patients with cPAN should be monitored closely over time for progression to systemic PAN. Progression of cPAN to systemic PAN is rare but has been reported.

The clinical features, diagnosis, and treatment of cPAN will be reviewed here. Systemic PAN and the general approach to the diagnosis of vasculitis are reviewed separately. (See "Clinical manifestations and diagnosis of polyarteritis nodosa in adults" and "Treatment and prognosis of polyarteritis nodosa" and "Overview of and approach to the vasculitides in adults" and "Evaluation of adults with cutaneous lesions of vasculitis".)

EPIDEMIOLOGY

Polyarteritis nodosa (PAN) is itself a rare disease, with an estimated prevalence of roughly 31 cases per million [2]. cPAN is an even rarer subset of PAN for which the true prevalence is unknown [3]. cPAN may account for approximately 4 percent of PAN cases [4].

                   
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Literature review current through: Sep 2017. | This topic last updated: Jan 01, 2017.
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