Langerhans Cell Histiocytosis Flashcards
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.
The histiocyte is a tissue macrophage or a dendritic cell (histio, diminutive of histo, meaning tissue, and cyte, meaning cell).
Langerhans Cell Histiocytosis- Fx
Features
bone pain, typically in the skull or proximal femur
cutaneous nodules
recurrent otitis media/mastoiditis
tennis racket-shaped Birbeck granules on electromicroscopy
Langerhans Cell Histiocytosis - Skull XR
SEE PASSMED IMAGE
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.
Langerhans Cell Histiocytosis - Example Question
A 7-year-old girl who has recently emigrated from Turkey is brought to the Emergency Department with pain on walking and multiple swellings over her head. She also complains of persistent headaches which are now quite distracting and causing her to miss school. This symptoms have been getting gradually worse for the past few weeks.
She has a past medical history of eczema and asthma which is well controlled with a salbutamol inhaler as required. There is no family history of similar problems.
On examination a number of soft tissue swellings are noted on the scalp. She also has non-specific tenderness over the proximal part of the left femur.
A skull x-ray is requested:
PASSMED SEE IMAGE
What is the most likely diagnosis?
Multiple myeloma > Langerhans cell histiocytosis Neutrofibromatosis Systemic mastocytosis Wiskott-Aldrich syndrome
The age of the patient combined with ‘punched out’ osteolytic skull lesions make a diagnosis of Langerhans cell histiocytosis likely.
Systemic mastocytosis results from a neoplastic proliferation of mast cells and is associated with urticaria pigmentosa and flushing.
