Rare. Due to overproduction of human growth hormone (hGH) in adults from a tumour (usually macroadenoma) gradual enlargement of bones of heads, hands, feet, and excessive overgrowth of soft tissues. F>M.
• Usually insidious onset 3rd and 4th decade of life.
• Earliest signs are coarsening of facial features and soft tissue swelling of feet and hands.
• Coarse body hair increases; skin thickens and often darkens. Increased sweating and sebum production. Protrusion of jaw.
• Peripheral neuropathies common (due to compression of nerves and endoneural fibrous proliferation – 40% have bilateral carpal tunnel).
• Cardiac disease and hypertension in up to 1/3rd
• 25% have glucose intolerance
• 50-70% have arthropathy – usually finger, spine and knees – joints enlarged, crepitus, effusions, increased mobility in early stages. Osteoarthritis is very common
• 50% usually have back ache
• 33% Raynauds phenomenon
• proximal muscle weakness – enzymes and EMG are normal
Based on clinical features and measure of plasma GH levels.
Complications – diabetes mellitus, hypertension, cardiomyopathy
Involvement of feet:
In early stages soft tissue swelling. Later on x-ray soft tissue thickening of toes; sesamoid bone enlarged; joint space widening; osseous proliferation at tendon and ligament attachments; skin thickens; excessive sweating
Calcaneal fat pad thickens – usually greater than 23mm (males) or 21.5mm (females).
Distal parathesias can occur
• surgical or radiation destruction.
• hGH levels in some patients may be lowered by bromocriptine mesylate.