Langerhans cell histiocytosis remains an enigmatic disease with a very heterogeneous presentation. We describe a rare case of orbital Langerhans cell histiocytosis in a 39-year-old female patient who presented right orbital pain and edema of the upper right eyelid. Surgery showed a friable lesion and underlying bone irregularity. Morphological aspects and immunohistochemical profile favored the diagnosis of Langerhans cell histiocytosis, which was confirmed with evidence of Langerin expression. The staging tests did not reveal any organ involvement, so we decided to follow the algorithm proposed by Euro Histio Net: in case of unifocal disease and in a single organ, clinical surveillance was preferred. This case aims to raise awareness of a manifestation of Langerhans cell histiocytosis, which should always be considered as a differential diagnosis in adults with osteolytic orbital lesions.
Background. Cogan's syndrome is a rare clinical entity whose etiopathology is still unknown, and the treatment strategies are not clearly defined. Case. A 23-year-old male presented with symptoms of headache, peripheral facial palsy, persistent right hearing loss and bilateral papillitis. Workup excluded all infectious, granulomatous, neoplastic, and immune causes. The diagnosis of atypical Cogan's syndrome was established, and the patient was treated with systemic corticosteroids and later on with cyclophosphamide and methotrexate. There were improvement of visual symptoms and stabilisation of left hearing. Conclusion. Cogan's syndrome is a very rare disease with no specific biological tests for the diagnosis. The diagnostic exams are mostly important to exclude other etiologies. The atypical ocular and audiovestibular manifestations make the diagnosis difficult, delaying the institution of appropriate therapy which may result in profound bilateral deafness.
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