• Elsa Berardi, Gianfranco Antonica, Annagrazia Procaccio, Donatello Marziliano, Nicola Susca, Patrizia Leone, Carlo Sabbà, Vito Racanelli, and Marcella Prete.
• Department of Interdisciplinary Medicine, Internal Medicine Unit, "Aldo Moro" University of Bari Medical School, Bari, Italy.
• Medicine (Baltimore). 2023 Dec 15; 102 (50): e36563e36563.

BackgroundCutaneous polyarteritis nodosa (cPAN) is a form of medium-sized vessel necrotizing vasculitis. It is a rare, skin-limited variant of polyarteritis nodosa, characterized by dermal and subcutaneous tissue involvement. The most common findings in cPAN include digital gangrene, livedo reticularis, and tender subcutaneous nodules. However, while limited to the skin, cPAN results in significant morbidity and mortality due to the accompanying skin ischemia and necrosis, such that patients are vulnerable to superinfection. Here, we describe a unique presentation of cPAN associated with pulmonary arterial hypertension (PAH).MethodsA 78-year-old female presented with digital ischemia and leg ulcers associated with PAH. Skin biopsy showed necrotizing fibrinoid necrosis of the small- and middle-sized vessels of the dermis. A diagnosis of cPAN and PAH was made. The patient was treated with glucocorticoids, vasodilators, and cyclophosphamide.ResultsShe died due to severe sepsis complications.ConclusionTo date, this is the first case report describing the association between cPAN and PAH. In this case, PAH is a complication of the cutaneous vasculitides suggesting that vasculopathy could play a role in the pathophysiology of PAH. However, the underlying pathophysiological mechanisms still have to be firmly established.Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.

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