Cutaneous polyarteritis nodosa and pulmonary arterial hypertension: An unexpected liaison. A case report.
Medicine (Baltimore)
; 102(50): e36563, 2023 Dec 15.
Article
en En
| MEDLINE
| ID: mdl-38115264
ABSTRACT
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.CONCLUSION:
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.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Poliarteritis Nudosa
/
Vasculitis
/
Enfermedades Cutáneas Vasculares
/
Hipertensión Arterial Pulmonar
Límite:
Aged
/
Female
/
Humans
Idioma:
En
Revista:
Medicine (Baltimore)
Año:
2023
Tipo del documento:
Article
País de afiliación:
Italia