背景:结节性皮肤多动脉炎(cPAN)是一种中型血管坏死性血管炎。这是一种罕见的,结节性多动脉炎的皮肤限制性变异,以真皮和皮下组织受累为特征。cPAN中最常见的发现包括数字坏疽,网状livedo,和皮下结节。然而,虽然仅限于皮肤,由于伴随的皮肤缺血和坏死,cPAN导致显著的发病率和死亡率,这样患者就容易受到双重感染。这里,我们描述了与肺动脉高压(PAH)相关的cPAN的独特表现。
方法:一位78岁的女性患者出现与PAH相关的指缺血和腿部溃疡。皮肤活检显示真皮中小血管的坏死性纤维蛋白样坏死。诊断为cPAN和PAH。患者接受糖皮质激素治疗,血管扩张剂,和环磷酰胺.
结果:她死于严重的脓毒症并发症。
结论:迄今为止,这是首例描述cPAN和PAH之间关联的病例报告.在这种情况下,PAH是皮肤血管炎的并发症,表明血管病变可能在PAH的病理生理学中起作用。然而,潜在的病理生理机制仍需牢固建立。
BACKGROUND: 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).
METHODS: 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.
RESULTS: She died due to severe sepsis complications.
CONCLUSIONS: 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.