University Library System, University of Pittsburgh
Doi
Abstract
Background: Tolosa-Hunt Syndrome (THS) is a rare inflammatory disorder presenting with painful ophthalmoplegia due to granulomatous involvement of the cavernous sinus or superior orbital fissure. Though autoimmune diseases such as systemic and discoid lupus erythematosus (SLE/DLE) are known to overlap with other disorders, their association with THS remains poorly documented. DLE, a chronic photosensitive condition with scarring skin lesions, is particularly rare in conjunction with THS. This report explores a case of THS associated with DLE, highlighting diagnostic complexities and therapeutic strategies.
Case: A 54-year-old woman presented with right-sided headache, diplopia, and ocular pain. Examination revealed right third cranial nerve palsy, hyperpigmented macular lesions, and alopecia areata. Imaging demonstrated cavernous sinus inflammation, and laboratory findings included elevated ESR, positive dsDNA titers, and a homogeneous immunofluorescence pattern. A biopsy confirmed DLE, aligning with clinical and imaging findings of THS. The patient was treated with corticosteroids and mycophenolate mofetil, resulting in sustained symptom resolution with no relapse during follow-up.
Conclusion: This case underscores the need to consider autoimmune conditions like DLE in patients with THS, suggesting a possible shared autoimmune mechanism. Early recognition and timely initiation of immunosuppressive therapy with corticosteroids and mycophenolate mofetil were key to achieving remission, supporting their use as first-line treatment. This report adds to the limited literature on DLE-associated THS and highlights the importance of thorough diagnostic evaluation and long-term follow-up to monitor progression and prevent recurrence. Additional reports are needed to improve understanding of the pathophysiology, clinical features, and optimal management of these rare coexisting conditions
