Langerhans cell histiocytosis
Langerhans cell histiocytosis is a rare condition associated with the abnormal proliferation of histiocytes. It typically presents in childhood with bony lesions.
Features
Features
- bone pain, typically in the skull or proximal femur
- cutaneous nodules
- recurrent otitis media/mastoiditis
- tennis racket-shaped Birbeck granules on electromicroscopy
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Young girl with multiple well defined 'punched out' osteolytic lesions with scalloped edges (geographic skull) are seen in the bilateral parietal regions. The lesions have a characteristic bevelled edge.