Case report: a 55 old woman with preudotumor cerebri, urticarial vasculuitis and suspected Sjogren syndrome

Abstract

Case report: We report a case of a 55 old woman presenting complaints of severe and permanent headache with progressive decrease of visual acuity (VA) for 6 months, xerophtalmia, xerostomia and urticarial eruption. The medical history of the patient is marked by autoimmune thyroiditis and active tobacco smoking (10 cigarettes / day during 20 years). The physical examination was remarkable by a normal body mass index and skin lesions specific for urticarial vasculitis. The remainder of examination was normal. The CBC, biochemical, auto-antibodies and radiological examinations were normal too. The evaluations included the assessment of the cephalalgia: complete neurologic examination, fundus examination, MR1 of the brain and a lumbar puncture. The results have revealed a papilledema, a decrease of VA and an elevation of the opening pressure at the lumbar puncture. The diagnosis conclusion was the presence of pseudotumor cerebri, which is a disorder clinically manifested by chronically elevated intracranial pressure of unknown etiology associated with visual abnormalities. The skin biopsy revealed small vessels wall deposits of IgG and C3, and a polynuclear perivascular infiltrate. The last question was the etiology of the Sicca syndrome, who is supposed to be a Sjogren syndrome. The Shirmer test and the salivary gland scintigraphy confirmed our suspicion. We proceeded to salivary gland biopsy, whose result is on pending. Conclusion: We report a case of a smoking woman who presents a pseudotumor cerebri, urticarial vasculutis where was suspected Sjogren syndrome, defined as a systemic chronic inflammatory disorder characterized by lymphocytic infiltrates in exocrine glands. If the principle of parsimony is used, the three entities can be linked: to our known, in the medical literature where are few case reports concerning the association of Sjogren syndrome and pseudotumor cerebri as a very rare neurological complication. In its turn, the urticarial vasculitis is a known manifestation of Sjogren syndrome.