A curious cause of bilateral upper extremity lymphedema: ‘Puffy hand syndrome’

A 36-year-old female with a history of illicit drug use presented for evaluation of progressive painless hand and finger swelling of 9 months’ duration. She related 2 years of repeated upper extremity intravenous injections of heroin and cocaine. When intravenous access could not be obtained, the dermal or subcutaneous regions of her dorsal hands and forearms were injected. Abstinence was achieved and maintained for the preceding 12 months. Other than multiple episodes of cellulitis and superficial venous thrombosis of the distal upper extremities, her medical and surgical history was otherwise unremarkable. Physical examination was only notable for relatively symmetric nonpitting edema within her dorsal hands and fingers (Panel A). Less severe edema affected the distal forearms. No lower extremity swelling was identified. Comprehensive laboratory analysis including testing for autoimmune and infectious diseases was unrevealing. Venous duplex ultrasonography of her upper extremities documented several bilateral hypoplastic/contracted superficial forearm veins with post-thrombotic changes but no acute superficial or deep venous thrombosis. Plain film hand radiography and computed tomographic venography of her chest were normal.

Panel A.

The patient was ultimately diagnosed with ‘puffy hand syndrome’ (PHS), an often overlooked complication of intravenous drug abuse affecting between 7% and 16% of addicts. ¹ In a case-controlled study of 66 patients, multivariate analysis identified the following risk factors for PHS: (1) female sex (odds ratio (OR) = 8.9, p = 0.03); (2) absence of tourniquet (OR = 7.0, p = 0.02); (3) injections into the hands (OR = 5.9, p = 0.03). ²

The pathophysiology is likely multifactorial and includes repetitive superficial venous and venular thrombosis (phlebolymphedema), recurrent soft tissue infections, and lymphatic obliteration from injections of the drug and its potentially impure adjuvants. ³ The diagnosis is typically made clinically, although corroborative lymphoscintigraphy may be required. No specific therapy for PHS exists other than supportive measures such as drug abstinence, skin care, and multimodal compression.

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