A 69-year-old woman presented with an 18-month history of progressive facial asymmetry, the right eye appearing more prominent than the left. She also complained of tearing of the right eye and inability to close the eye tightly. She had noted recurrent focal superficial red skin lesions on the right brow that initially blistered and then healed over 2-3 weeks. She described similar lesions occurring on her hands and lower legs over the past 2 years that resolved after local steroid cream treatment prescribed by her primary care physician. The patient denied any disturbance in her vision, headaches, or other neurologic symptoms. Hearing was normal. Her medical history was unremarkable, and she was taking no current medication. She had smoked 15-20 cigarettes a day for 50 years. She had lived and worked in Lombok, Indonesia, for 8 years, returning to live in the United Kingdom 3 years before presentation.On examination, the patient's best-corrected visual acuity was 20/20 bilaterally with normal visual fields. Extraocular movements were full, and the patient was orthophoric. There was no proptosis. The anterior and posterior segments of each eye were normal. There were foci of erythema and blistering on the right brow ( Fig. 1A). Right facial weakness was evident and involved the orbicularis oculi disproportionately (Fig. 1B). Left facial nerve function was normal. After forceful bilateral eye closure, mid-facial fasciculations lasting 5-10 seconds were seen on the right side. Trigeminal sensory function was reduced in the first and second divisions on the right, including corneal sensation; motor function was normal. Bell phenomenon was preserved, and all other cranial nerve function was intact.Routine hematologic and biochemical studies, including liver function tests, revealed no abnormalities. Serum immunoglobulins were normal as were inflammatory markers, including eosinophil sedimentation rate and c-reactive protein. The antinuclear antibody test and an autoantibody screening were negative. Varicella zoster virus-specific IgM antibody was negative as were both Lyme and treponemal serologies. Chest radiography was normal. MRI revealed no abnormalities other than agerelated involutional changes and T2 hyperintensities consistent with microvascular disease in the deep white matter of both cerebral hemispheres. The orbits were normal. There was no space-occupying or inflammatory lesion in the pons, and both facial nerves and trigeminal nerves had normal imaging characteristics.The patient's clinical signs gradually progressed. The right facial palsy increased, and she developed a right lower lid entropion. She also developed numbness in the distal lower limbs and increased frequency and persistence of the skin lesions. Neurological examination showed reduced sensation to temperature and touch below the knees and an anesthetic patch on the left palm. Knee flexion and extension were weak. The patient was referred for a dermatological assessment.
Drs Venning and Parks:Dermatological examination revealed that the fa...