ACROANGIODERMATITIS: A RARE CUTANEOUS MANIFESTATION OF CENTRAL VEIN STENOSIS

 

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ACROANGIODERMATITIS: A RARE CUTANEOUS MANIFESTATION OF CENTRAL VEIN STENOSIS

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Rizky
Andhika
Rizky Andhika rizkyandhikaipd@gmail.com Dr. Hasan Sadikin General Hospital, Faculty of Medicine Universitas Padjadjaran Division of Nephrology and Hypertension, Department of Internal Medicine Bandung Indonesia *
Afiatin Makmun afiatinmakmun@gmail.com Dr. Hasan Sadikin General Hospital, Faculty of Medicine Universitas Padjadjaran Division of Nephrology and Hypertension, Department of Internal Medicine Bandung Indonesia -
Ria Bandiara riabandi81@gmail.com Dr. Hasan Sadikin General Hospital, Faculty of Medicine Universitas Padjadjaran Division of Nephrology and Hypertension, Department of Internal Medicine Bandung Indonesia -
Rudi Supriyadi rudisdoc@gmail.com Dr. Hasan Sadikin General Hospital, Faculty of Medicine Universitas Padjadjaran Division of Nephrology and Hypertension, Department of Internal Medicine Bandung Indonesia -
Lilik Sukesi liliksukesi@gmail.com Dr. Hasan Sadikin General Hospital, Faculty of Medicine Universitas Padjadjaran Division of Nephrology and Hypertension, Department of Internal Medicine Bandung Indonesia -
Rizqa Adinda Syakira rizqadindasyk2@gmail.com Dr. Hasan Sadikin General Hospital, Faculty of Medicine Universitas Padjadjaran Division of Nephrology and Hypertension, Department of Internal Medicine Bandung Indonesia -
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Vascular access is a fundamental element in hemodialysis therapy. Even though an Arteriovenous Fistula (AVF) is preferable, the use of a central venous catheter (CVC) is often inevitable. CVC use may lead to long-term complications such as central vein stenosis (CVS). CVS encompasses a wide range of clinical presentations from asymptomatic to severe superior vena cava syndrome due to central vein occlusion. This case reports a hemodialysis patient with CVS presenting a rare cutaneous manifestation of Acroangiodermatitis (AAD) secondary to venous hypertension.

A 53-year-old man with end-stage kidney disease (ESKD) on hemodialysis for the past 16 years, presented with an extensive crusted ulcer accompanied by edema extending from right shoulder to the fingers, along with venectasia around the right AVF cannulation site. The symptoms had been progressing for eight months, initially presenting as swelling of the right arm extending to the chest, which worsened particularly after hemodialysis sessions. The patient underwent several methods of vascular access with temporary CVC insertions in the right neck, AVF in the left lower and upper arm, before establishing a radiocephalic fistula in the right lower arm for the past eight years. Angiographic evaluation revealed a right subclavian vein total occlusion that was impenetrable by a guidewire. Accordingly, new vascular access was created, leading to partial improvement of the edema. Four months later, ulceration with yellowish discharge developed at the previous AVF cannulation site, evolving into thick enlarged crusted plaques unresponsive to topical antibiotics. On examination, the patient was hypertensive with conjunctival pallor, elevated jugular venous pressure (JVP), and venectasia over the anterior thoracic wall. The right arm showed hyperpigmented erythematous plaques with ulceration and overlying crusts. Acroangiodermatitis was diagnosed based on dermoscopic and histopathological findings. The patient underwent percutaneous transluminal angioplasty (PTA), followed by a planned wide excisional necrotomy, AVF ligation, intraoperative biopsy, and defect closure with a split-thickness skin graft.

Figure 1. Clinical PresentationFigure 2. Right Subclavian Vein Occlusion

Central vein stenosis (CVS) is a major cause of venous hypertension in patients with AVF. CVC insertion and long-standing AVF are some risk factors of CVS. Venous hypertension rarely presents with dermatologic manifestations such as acroangiodermatitis (AAD) resulting from reactive proliferation of dermal vessels. Management of AAD in hemodialysis patients primarily focuses on addressing the underlying vascular obstruction. In this case, the chosen therapeutic strategy consisted of percutaneous transluminal angioplasty (PTA) as the recommended initial therapy, followed by a planned wide excisional necrotomy, right arteriovenous fistula (AVF) ligation, intraoperative biopsy, and defect closure with a split-thickness skin graft to preserve the affected limb.

This case highlights a complex vascular access complication in a hemodialysis patient that required a multidisciplinary approach. Early recognition and prompt management of central vein stenosis and its complications, including acroangiodermatitis, are crucial to optimize patient outcomes.

Kewords