Chronic idiopathic hyperphosphatasemia

Chronic idiopathic hyperphosphatasemia, or juvenile Paget disease is a very rare syndrome that is characterized by fragile bones, bowing deformities, shortness of stature, large head, premature loss of teeth, radiographic evidence of expanded osteoporotic long bones with coarse trabeculations, and widened bones of the skull. Increased levels of serum alkaline phosphatase and increased levels of urinary total hydroxyproline are notable. We present a case of juvenile Paget disease that was associated with a history of precocious puberty. The patient had odontogenic osteomyelitis of the mandible that was treated by drainage, surgical debridement and antibiotic therapy.