Spontaneous intracranial hypotension syndrome in systemic sclerosis.
A 52-year-old woman was diagnosed as having limited cutaneous systemic sclerosis (lcSSc) in June 2003. The cutaneous impairment of systemic sclerosis (SSc) was characterized by sclerodactyly, telangiectasia, pitting scars and calcinosis cutis. Systemic manifestations of lcSSc included: Raynaud's phenomenon and esophageal impairment characterized by low pressure in the lower esophageal sphincter and decreased peristalsis in the lower two-thirds of the esophageal body on manometry. Autoantibody screening tests were positive for antinuclear antibodies (1 : 1200) with anticentromere antibody. Other tests, including pulmonary function tests (PFT), computed tomographic (CT) scan of the lungs and echocardiography, were within normal limits. She received therapy with combined diltiazem and omeprazole.

In October 2009, the patient presented with a 1-month history of daily severe headache, which was refractory to common analgesics. The headaches worsened abruptly upon standing and improved in the supine position. On admission, the patient had no fever. There was no nuchal rigidity and neurological examination was normal. General physical examination revealed necrotic digital ulcers involving the second and third left fingertips. Laboratory studies disclosed the following: C-reactive …