Atypical Sjögren presentation of pruriginous rash and mild eosinophilia with minor sicca symptoms: A case report

Abstract

We report a case of an 82-year-old woman diagnosed with Sjögren syndrome on the basis of salivary gland biopsy and anti-Ro/SSA antibodies who presented to our institution with a pruriginous maculopapular pancorporeal rash with diffuse eosinophilic infiltrate and mild eosinophilia. Sicca symptoms were mild. Cutaneous and hematologic findings resolved spontaneously within 4 weeks, the rash did not respond to antihistamines but was mildly attenuated by topical corticosteroid. Both the rash and hypereosinophilia greatly improved while the patient received oral corticosteroids for a contrast medium allergy before a CT-scan. When the patient was diagnosed with SS, hydroxychloroquine treatment was introduced. This case report aims to raise awareness of the possibility of such a clinical presentation, considering the known increased risk of non-hodgkin lymphoma associated with Sjögren syndrome estimated to 5%-10% lifetime risk which is 5 to 44 times higher than that of the general population.