Intestinal and neurological involvement in Behcet disease: a clinical case

Abstract

BackgroundBehcet’s disease (BD) is a chronic immune-mediated, inflammatory disorder which may affect a number of different systems (oral and genital mucosa, eyes, skin, vascular district, joints, gastrointestinal tract and nervous system). Neurological manifestations are present in 5–10%, and gastrointestinal tract involvement in 10–15% of cases. The simultaneous involvement of two systems, neurological and gastrointestinal tract, is very rare and represents the aim of our case report.Case presentationWe describe a case of a 12-year-old girl with neurological (endocranial hypertension, papilledema, retinal vasculitis) and gastrointestinal tract (terminal ileum and cecum inflammation) involvement and with a history of recurrent oral aphthosis; therefore, according to both International Criteria for Behcet’s Disease (ICBD) and Paediatric Behcet’s Disease criteria (PEDBD) the diagnosis of BD was confirmed.ConclusionsThis case report is one of the few described in literature with simultaneous involvement of the two systems, neurological and gastrointestinal tract, in paediatric BD. The diagnosis is really difficult because there is no specific diagnostic test. We think that our clinical case should help clinicians to suspect a BD with an unusual onset.

Behcet’s Disease with Upper GI Bleeding

“…Behcet's disease can reach serious proportions without early diagnosis and treatment [2,7,8]. Almost all patients of BD present with lesions of painful nonscarring oral mucocutaneous aphthous ulcerations [3,5]. Eye involvement with scarring and bilateral panuveitis is the most serious complication which occasionally progresses rapidly to blindness if untreated early [3].…”

“…e prevalence of Behcet's disease in Ethiopia is not known, and only one case was reported from Ethiopia in 1997 [2]. Usually, BD is diagnosed in young adults (20-35 years), with no difference between sexes [5].…”

Treatment Options in Pediatric Behçet’s Disease

“…Terminal ileum and ileocecal regions are typically affected by lesions that have the aspect of aphtae or deeper volcano-shaped ulcers with deep and defined margins and a focal distribution. The terminal ileum and ileocecal regions are typically affected by lesions that have the aspect of aphthae or round/oval-shaped ulcers with deep and defined margins and a focal distribution [ 51 – 54 ]. Arterial involvement may result in intestinal ischemia.…”