STEWART–BLUEFARB SYNDROME: A RARE CASE AND REVIEW

Stewart-Bluefarb syndrome also known as acroangiodermatitis or Kaposi's Pseudosarcoma, is a rare disorder encountered that falls under the broader category of skeletal dysplasia’s, its precise etiology is still under active investigation. In patients undergoing hemodialysis, this syndrome can manifest as a secondary condition resulting from an underlying arteriovenous malformation. Here, we report a clinical case illustrating this uncommon condition.

Case report

A 57-year-old male with a history of hypertension and end-stage renal disease (ESRD) required vascular access for long-term hemodialysis. A right radiocephalic fistula (RCF) was initially constructed, but it failed early. Consequently, a left brachiocephalic fistula (BCF) was created, which remained functional for one year. After initiation of BCF use, the patient developed mild edema in the left forearm, which worsened, leading to superior vena cava syndrome. First tomography angiography (CTA) revealed stenosis of superior vena cava and the left innominate vein. Vascular surgery attempted angioplasty without success. Given the limited vascular access options, hemodialysis via the BCF was continued.

Fourteen-months after central vessel stenosis diagnosis, the patient developed progressive, ulcerated, plaque-type lesions on the dorsal region of the hand and left forearm, measuring approximately 10x7 cm. The lesions were scabbed and bleeding, suggestive of superimposed infection (Figure 1). Culture analysis yielded positive results for Escherichia coli, followed later by Pseudomonas aeruginosa Nevertheless, broad-spectrum antibiotic therapy with carbapenem and glycopeptide was initiated based on susceptibility results.

The cephalic vein showed tortuosity, collaterals, and a 74% occlusive thrombus in its distal third. Doppler ultrasound of the left upper limb showed peak systolic velocities (PSV): 203 cm2 /sec, blood flow: 3654 ml/min (Figure 2). A follow-up CTA revealed complete proximal occlusion of the left internal jugular and brachiocephalic veins, 25% stenosis of the right brachiocephalic trunk, and segmental stenosis (40% proximal, 20% distal) of the superior vena cava (Figure 3).

Following a skin biopsy, a diagnosis of fragmented keratoacanthoma was established, ruling out malignant neoplastic lesions. Histological findings were consistent with lesions secondary to necrosis, suggestive of Kaposi's Pseudosarcoma (Figure 4). The patient underwent left upper limb debridement, followed by left innominate vein angioplasty and reconstruction of the right RCF (functional access) thereafter left BCF with collateral veins, was surgically closed.

The occurrence of Stewart-Bluefarb syndrome as a complication of arteriovenous fistulas for hemodialysis is exceptionally rare, with no reported prevalence, partly due to its complexity, potential under-recording, and a lack of clinical suspicion. Although clinical evaluation remains pivotal, histopathological and microbiological analyses are essential to exclude malignancies such as Kaposi’s sarcoma or squamous cell carcinoma. Ultimately, the lesions ulcerated and bled due to infection, requiring targeted antibiotic therapy multiple surgical interventions, including closure of the arteriovenous fistula, these combined interventions served as definitive treatment and full recovery within 90 days.