A Rare Case of Langerhans Cell Histiocytosis of the Skull in an Adult: a Systematic Review
A 41-year old male who presented to the Emergency Department after falling while water-skiing at 40-50 km/hr. He landed face first onto the water but did not lose consciousness and remembered that he was in trouble.\nThe patient recalls his face feeling puffy for 10-15 min but then settled and both his eyes were bleeding for approximately 30 min. He complained of neck and right shoulder pain and a frontal headache. His previous medical history included surgery for lumbar disc prolapse and chronic headaches, which had persisted for the last 2-3 months prior to presentation. Medications included Dothiepin (TCA) and Salazopyrin (Sulfasalazine). On assessment, the patient was conscious (GCS 15), had a BP of 123/92, pulse of 72, and an oxygen saturation of 95%. There was periorbital bruising and swelling with a laceration of the right inner aspect of the upper eye-lid which was bleeding and blood was present in both eyes. A full opthalmology assessment found no abnormalities. Radiological assessment included X-rays of the head (), pelvis, C-spine, right shoulder, lumber spine, and a CT of the head. The radiographs showed no evidence of acute fractures/dislocations. The head CT showed a small hypersensitivity with a small extra axial collection with a maximum thickness of 1mm. Differential diagnoses included an arachnoid cyst, haemangioma, meningioma or a secondary lesion. Due to the possibility of a malignant lesion a bone scan was also undertaken and subsequently showed an active lesion. The patient was admitted and had a craniotomy to excise the skull tumor. The excised lesion consisted on bone with soft tissue (30×20×7 mm). Histopathological examination of the specimen, which included immunoperoxidase studies revealed a rounded defect of the bone which consisted of a proliferation of fibrous stroma within which can be seen inflammatory foci ().\nImmunoperoxidase studies were positive for S100 and CD68 and negative for HMB45 and Cam 5.2. The patient is currently well.