Atypical Presentation of Langerhans Cell Histiocytosis of th
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A man in his 20s presented with a 4-week history of progressive swelling of the midline forehead, recurrent headache, and sinus pain. Over the same time period, he reported concurrent bacterial sinusitis recalcitrant to multiple antibiotic regimens including amoxicillin and doxycycline.

Physical examination was notable for a protuberant, tender, mobile tumefaction of the forehead (6 × 4 cm). Findings of CT imaging demonstrated a 1.7-cm lytic lesion of the left frontal bone with mucosal thickening of the left frontal sinus. MRI of the brain revealed a bilobed 1.9 × 2.4-cm heterogeneously enhancing lesion at the site of the lytic lesion with extension extracranially into the subperiosteal tissues as well as intracranially with thickening and enhancement of the underlying meninges.

On the basis of his clinical presentation, frontal sinusitis with osteomyelitis and abscessualization phenomena (ie, Pott puffy tumor) was suspected. However, findings on radiographic imaging did not demonstrate central necrosis consistent with abscess. Nevertheless, treatment with broad-spectrum antibiotics was initiated followed by operative debridement.

A bicoronal skin incision was taken down extending past the superior temporal line. The soft-tissue lesion was visualized and sent for analysis. Abnormal-appearing bone surrounding the skull defect was craniectomized to evacuate phlegmon extending intracranially through the abnormality. A small breach within the right frontal sinus mucosa was identified for which it was opened and exenterated. Histopathologic analysis of the soft tissue and bone demonstrated a leukocytic infiltrate as well as histiocytes, multinucleated cells, and Langerhans cells. As such, the microscopic and immunophenotypic elements of Langerhans cells in an osteolytic lesion led to a diagnosis of Langerhans cell histiocytosis (eosinophilic granuloma).

This case represents the first report of Langerhans cell histiocytosis of the skull presenting as frontal sinusitis in an adult. We emphasize that Langerhans cell histiocytosis should be considered in the differential diagnosis for patients with complex lesions of the skull and overlying soft tissues. Moreover, in such cases, biopsy of the osteolytic lesion should be routinely performed to avoid misdiagnosis and guide appropriate treatment.

Source: https://jamanetwork.com/journals/jamaotolaryngology/fullarticle/2770085
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