BACKGROUND: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder of unknown etiology which can present at any age with symptoms of mucocutaneous, musculoskeletal, renal, central nervous system, and nonspecific clinical pictures making the disease a “master of mimicry”.

CASE REPORT: A 53-year-old female, who was recently diagnosed with SLE, presented with right-sided sharp and electric shock-like facial pain starting at the side of her right nostril and traveling down the naso-labial fold and then back to the angle of the jaw, mostly in the region of V2–V3 distribution with no radiation beyond trigeminal distribution. Her pain had been going for the last 2 years and was regarded as “pretrigeminal neuralgia”; however, it progressed in frequency over the last 2 weeks, with no clear identifying triggering factors. Her laboratory test results showed positive anti-nuclear antibodies (ANA) with raised titer, anti-double-stranded DNA, anti-ribonucleoprotein, anti-Sjögren’s syndrome-related antigen A, anti-Sjögren’s syndrome-related antigen B, and anti-smooth muscle antibodies. Other possibilities of migraine, postherpetic neuralgia, Bell’s palsy, and brain tumor were ruled out. A diagnosis of SLE with trigeminal neuralgia (TN) was made and carbamazepine 100 mg 2 times a day was prescribed.

CONCLUSIONS: TN is seldom mentioned as a neurological manifestation of SLE; hence, we recommend further studies to investigate the SLE-mediated injury to trigeminal fibers to make a timely diagnosis of TN and to prevent progressive autoimmune process-related vasculitic and demyelinating changes.

Keywords: Autoimmune Diseases, Lupus Vasculitis, Central Nervous System, Trigeminal Neuralgia