Involvement of the jaw bones in systemic mastocytosis.
Systemic mastocytosis is characterised by proliferation of mast cells and infiltration of organs. Severe bony pain may result from release of chemical mediators from mast cells and affected patients are at an increased risk of anaphylaxis. Traditional analgesics such as non-steroidal anti-inflammatory agents and opioids are contraindicated. Diagnosis is based on presentation, biopsy of bone marrow, and magnetic resonance imaging of the affected area. In the head and neck, the disease may present as facial pain, localised osteomyelitis, oral sinus formation, and oral ulceration. Treatment is with histamine antagonists and bisphosphonates to control symptoms. An adrenaline pen is provided for use in case of anaphylaxis.
Castling
B
B
Derby Royal Infirmary, London Road, Derby DE1 2QY, UK. bcastling@yahoo.co.uk
Smith
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AT
Myers
B
B
D000328:Adult; D050071:Bone Density Conservation Agents / Q000627:therapeutic use; D004164:Diphosphonates / Q000627:therapeutic use; D006634:Histamine H1 Antagonists / Q000627:therapeutic use; D006801:Humans; D007573:Jaw Neoplasms / Q000188:drug therapy*; D008297:Male; D034721:Mastocytosis, Systemic / Q000188:drug therapy*; D000077268:Pamidronate; D012888:Skull Neoplasms / Q000188:drug therapy
D050071:Bone Density Conservation Agents; D004164:Diphosphonates; D006634:Histamine H1 Antagonists; D000077268:Pamidronate