Hyperphosphatasemia in an adult. Clinical, roentgenographic, and histomorphometric findings and comparison to classical Paget's disease

A 54-year-old man with short stature, diffuse skeletal abnormalities, and elevated alkaline phosphatase was evaluated by bone biopsy with undecalcified sections and morphometry analysis. Microscopically the bone changes were identical to those of classic Paget's disease. Histomorphometric analysis of bone demonstrated a high remodeling activity with increased mineralization rate similar to Paget's disease. However, the early age at onset and severity suggest that this man suffers from hyperphosphatasemia, a different and distinct condition.