Puffy Hand Syndrome: What Plastic Surgeons Need to Know

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A 26-year-old female with a remote history of intravenous (IV) drug abuse presented to the emergency department with unilateral swelling and acute pain of her left hand. She was found to have diffuse bullae and erythema of her left hand (Figure 1A). After imaging, the patient was taken to the operating room due to concern for possible necrotizing soft tissue infection (NSTI). There was no evidence of devitalized tissues or purulence on exploration; however, significant tissue edema was noted. She was treated for cellulitis with IV antibiotics and transitioned to oral antibiotics based on intraoperative cultures.

Figure 1. Preoperative images from the first and second presentations.

The patient returned 1 month later with swelling of the contralateral hand; however, she had more extensive bullae and swelling, with erythema of the upper extremity (Figure 1B). Pain with passive motion was noted. Given that her symptoms and clinical findings were concerning for NSTI, she was taken to the operating room for exploration. Once again, significant tissue edema but no gross infection was found. (Figure 2A).

After ruling out NSTI, puffy hand syndrome with superimposed cellulitis was diagnosed. The patient was treated with elevation, compression, and targeted antibiotic therapy. See Figure 2B for a postoperative image.

Figure 2. Intra- and postoperative images from the first and second presentations.

Puffy hand syndrome is a complication of IV drug abuse first described in prisoners in New York in 1965 by Abeles.¹ Patients first suffer from intermittent painless edema of the hand that can begin during or after a period of IV drug abuse. Eventually there is progression to permanent edema unresponsive to elevation. The edema is either non-pitting or minimally pitting, affecting the dorsal surface of the fingers and hands, sometimes asymmetric, with skin thickening.² The pathophysiology of the disease is not entirely understood but is thought to be from the sclerosing of the superficial venous system and the lymphatics from IV injections of irritants commonly found in street drugs or insoluble compounds such as buprenorphine. This complication can affect up to 16% of IV drug users.³

In a patient presenting with unilateral or bilateral swollen hands, a full history should be taken with special attention to previous surgical history or history of IV drug use. A physical exam should be conducted with attention to the vascular exam, symmetry of the upper extremities, skin coloration, and quality of the edema. In patients with puffy hand syndrome, the edema will be non-pitting, affecting the dorsum of the hand, and the extremity may have thickened skin.² The differential diagnoses for puffy hand syndrome include lymphangitis, venous thrombosis, heart failure, renal insufficiency, liver failure, erythromelalgia, scleroderma, rheumatoid arthritis, and systemic lupus erythematosus.

When acute pathology has been ruled out in patients with a history of IV drug abuse, the diagnosis of puffy hand syndrome can be made. Presentation can be acute, such as in cases of cellulitis, or with complaints of longstanding swelling of the bilateral hands.^2-5 Lymphoscintigraphy, demonstrating lymphatic blockage, can be an objective means to help confirm diagnosis but is not necessary.⁶

Differentiation of acute superimposed cellulitis from NSTI is critical. NSTI is the most feared presentation of acute upper extremity infection, as it is associated with high morbidity and mortality rates. Clinical suspicion for a patient with a presentation such as the one highlighted in this case should be high, as NSTI can present similar to cellulitis in its early stages. Areas of cellulitis should be marked and reexamined within 1 hour, with rapid progression helping to distinguish NSTI from cellulitis.⁷ The Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) scoring system is a useful tool for surgical decision-making.⁸ However, the ultimate decision for operative exploration is based on clinical suspicion. Intraoperatively, the ability to easily pass an instrument between the fascia and intact skin would be a characteristic finding of NSTI that is not seen in cellulitis.⁷

Swelling is a lifelong complication of puffy hand syndrome, and currently there are no therapies, medical or surgical, to reverse its course. There are surgical treatments for lymphedema, including lymphaticovenous anastomosis, vascularized lymph node transfer, and suction-assisted protein lipectomy.⁴ However, these techniques have not been used in the treatment of puffy hand syndrome. Treatment is currently limited to the symptomatic management of the swelling with low-stretch bandages and elevation.⁵

Puffy hand syndrome has a predictable course in that it begins with intermittent swelling and progresses to permanent swelling after several months. The presentation of the disease can be variable in its temporal relationship to IV drug abuse, which may be remote or ongoing.² The swelling is a lifelong complication, and currently there are no therapies, medical or surgical, to reverse its course.