Case report: Budesonide alleviated recurrent ulcerative stomatitis and pneumonia of pediatric Behcet-like disease

Read the full article See related articles

Discuss this preprint

Start a discussion What are Sciety discussions?

Listed in

This article is not in any list yet, why not save it to one of your lists.
Log in to save this article

Abstract

Background Behcet’s disease (BD) is an idiopathic, chronic, relapsing, multi-systemic vasculitis characterized by recurrent oral and genital aphthous ulcers, ocular disease, skin lesions, venous thrombosis and arterial pseudo- aneurysms. Behcet-like disease (BLD) refers to conditions that mimic the clinical features of BD but do not fully meet the diagnostic criteria. Case presentation We report a girl who presented with Behcet’s-like phenotype including recurrent aphthous stomatitis, peumonia and pulmonary venous thrombosis, diagnosed with BLD due to the c.951C>A (p.H317G) mutation in the C-C Chemokine Receptor 1(CCR1) gene. The patient suffered from intermittent fever, abdominal pain, diarrhea, oral ulcers, and weight loss, her clinical symptoms cannot be well controlled by conventional antibiotics, supportive care, immunosuppressants and biologics. Budesonide, a class of corticosteroid, was used to treat the patient and the patient's condition was significantly improved. Conclusion For children with BLD, individualized treatment should be carried out according to the clinical course of the patients. Budesonide can serve as an effective therapeutic drug option.

Article activity feed