Behçet’s disease (BD) is a multisystem recurrent vasculitic disorder of unknown origin, which manifests as recurrent oral and genital ulcers, skin and eye alterations. Hypertrophic pachymeningitis is a rare disease characterized by localized or diffuse thickening of the dura mater of brain associated with infections, systemic autoimmune/vasculitic disorders, malignancy and meningioma.
A 35-year-old gentleman presented with two-year history of bilateral trismus and jaw pain which did not resolve, despite oral surgery was done. He also had fever, headache, blurring of vision with restricted left eye movement for three months. CT Brain and Orbit with contrast showed extensive pachymeningeal enhancement, bilateral retrobulbar mass and right infratemporal fossa lesion. MRI of Brain and Orbit showed diffuse pachymeningeal enhancement and inflammation of left recti muscles, right infratemporal and masticator spaces. Patient was empirically covered with antibiotics for meninigitis. Subsequently, he was started on antituberculous treatment (anti-TB) due to high ESR and unresponsiveness to antibiotics. CSF results (cell count, biochemistry,C+S, acid-fast bacteria, MTB PCR, viral screening, GeneXpert) were not suggestive of infection. Despite being on anti-TB for eight months, fever did not resolve. Therefore, he was given trial of oral steroids (Tab Prednisalone) and his general condition improved. Due to worsening restriction of extraocular movement of left eye, left orbitotomy and retrorbital mass incisional biopsy were done. Left periorbital and orbital fat sent for histopathological examination showed neutrophilic vasculitis suggestive of Behcet’s disease. Currently, patient is started on Tab Azathioprine and being followed-up as outpatient.
DISCUSSION AND CONCLUSION
There should be high degree of suspicion about Behcet’s disease in patients with hypertrophic pachymeningitis with multisystem involvement although certain typical features such as recurrent oral and genital ulcerations are absent. Such atypical isolated cases has been reported worldwide. Early treatment with steroids are essential to halt progression of the disease and prevent serious complications.