Department of Interdisciplinary Medicine, Internal Medicine Unit, "Aldo Moro" University of Bari Medical School, Bari, Italy.
Centre for Medical Sciences, University of Trento and Internal Medicine Unit, Santa Chiara Hospital, Provincial Health Care Agency (APSS), Trento, Italy.
Medicine (Baltimore). 2023 Dec 15;102(50):e36563. doi: 10.1097/MD.0000000000036563.
Cutaneous 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).
A 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.
She died due to severe sepsis complications.
To 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.
皮肤型多发性动脉炎（cutaneous polyarteritis nodosa，cPAN）是一种中等大小血管坏死性血管炎。它是一种罕见的皮肤局限性多发性动脉炎，其特征是真皮和皮下组织受累。cPAN 最常见的表现包括指端坏疽、网状青斑和触痛性皮下结节。然而，尽管局限于皮肤，cPAN 仍会因伴随的皮肤缺血和坏死而导致显著的发病率和死亡率，使患者易发生继发感染。在这里，我们描述了一例与肺动脉高压（pulmonary arterial hypertension，PAH）相关的 cPAN 独特表现。
一名 78 岁女性因 PAH 出现指端缺血和腿部溃疡。皮肤活检显示真皮中小血管的纤维蛋白样坏死。诊断为 cPAN 和 PAH。患者接受了糖皮质激素、血管扩张剂和环磷酰胺治疗。
她因严重败血症并发症死亡。
迄今为止，这是首例描述 cPAN 和 PAH 之间关联的病例报告。在本例中，PAH 是皮肤血管炎的并发症，提示血管病变可能在 PAH 的病理生理学中起作用。然而，潜在的病理生理机制仍需进一步确定。
