A Pediatric Case of Bechet's Disease in a 14 Year Old Male: A Case Report

Document Type

Abstract

Publication Date

4-1-2020

Publication Title

Journal of Investigative Medicine

Volume

68

Issue

4

First page number:

948

Last page number:

948

Abstract

Purpose of Study: Behcet's disease is a primary variable vasculitis that may involve vessels of any size in both the arterial and venous system. This autoinflammatory process is rare in the general population with an estimated prevalence of 5-7 per 100 000 persons and has an even lower incidence in the pediatric population with only less than 10% of reported cases occurring in this population. It is classically described in the literature as recurrent oral ulcerations with skin findings and uveitis. While the underlying cause of Behcet's has not yet been elucidated, it is theorized that it is likely polygenic in nature with association with certain HLA subtypes such as HLA B51/B5. It is also theorized that a dysfunctional response to certain infectious microbes including bacteria and viruses may also play a role. It has been noted that the syndrome has a higher incidence in persons of Asian descent and is often more severe in this population.

Methods Used: In this case, we discuss one such case of this rare autoinflammatory disorder in a 14-year-old male with a two-year history of recurrent mouth ulcers with associated preceding fevers, body aches and new-onset painful genital ulcers.

Summary of Results: A 14-year-old male with a two-year history recurrent oral lesions which were initially attributed to HSV 1 and had been evaluated by adult infectious disease prior to his presentation to our institution. He had five episodes of recurrent stomatitis prior to presentation. The patient at the time of presentation had significant oropharyngeal involvement as well as genital involvement. He was febrile (104 F) and noted to have a mild leukocytosis at 18.1 k/mm3 with 85.7% neutrophils and a platelet count of 581 k/mm3. Further STI testing was obtained including HIV, GC (gonococcal) and Chlamydia all of which were negative. HSV PCR and culture were also obtained, and the patient was empirically started on acyclovir. This was discontinued when these resulted as negative as well. Due to the patient's extensive oral mucosal involvement, the decision was made to start PPN (Peripheral Parenteral Nutrition) and a second line had to be placed. This resulted in significant swelling of the upper extremity initially thought to be due to infiltration but later determined to be secondary to pathergy. Upon reaching the diagnosis of Behcet's the patient was started on high dose steroid therapy and had significant improvement of oral and genital lesions. He was subsequently transitioned to oral steroids and was able to be discharged home with close outpatient rheumatology and ophthalmology follow up.

Conclusions: Behcet's is a rheumatologic condition which, though rare, can cause significant morbidity if there is a delay in diagnosis including blindness secondary to ocular disease and amyloidosis secondary to persistent inflammation. Mortality in Behcet's may also occur secondary to its vascular involvement with pulmonary artery aneurysms and is as high as 25% in patients that develop this condition. It is imperative that providers are aware of this disease process, allowing for earlier diagnosis and treatment and in so doing preventing these sequelae.

Disciplines

Medical Specialties | Medicine and Health Sciences | Pediatrics

Language

English

UNLV article access

Search your library

Share

COinS