String-of-pearls : Extensive medium vessel vasculitis associated with childhood lupus

Abstract

Case details: A 16-year boy had a history of rash, oral ulcers, alopecia, photosensitivity, cheilitis, and weight loss, for which he was started on steroids, methotrexate, and hydroxychloroquine from outside. Three years later, he developed pericardial effusion and peripheral neuropathy, that were managed at an outside hospital. Later, he presented to us with weight loss, diffuse rash, left facial palsy, and left lateral rectus palsy. CT aortogram revealed a string-of-pearls appearance involving the head, neck, thoracic, and visceral arteries. The child was diagnosed with severe lupus vasculitis with minimal systemic symptoms. This child responded well to the methylprednisolone and cyclophosphamide.

Conclusions: This is a unique example of the classical multisystemic presentation of lupus and its rare manifestation of extensive systemic vasculitis.