Clinical Immunology Review Series: An approach to the patient with recurrent orogenital ulceration, including Behçet's syndrome

Abstract

ARTICLES PUBLISHED IN THIS CLINICAL IMMUNOLOGY REVIEW SERIES SummaryPatients presenting with recurrent orogenital ulcers may have complex aphthosis, Behçet's disease, secondary complex aphthosis (e.g. Reiter's syndrome, Crohn's disease, cyclical neutropenia) or non-aphthous disease (including bullous disorders, erythema multiforme, erosive lichen planus). Behçet's syndrome is a multi-system vasculitis of unknown aetiology for which there is no diagnostic test. Diagnosis is based on agreed clinical criteria that require recurrent oral ulcers and two of the following: recurrent genital ulcers, ocular inflammation, defined skin lesions and pathergy. The condition can present with a variety of symptoms, hence a high index of suspicion is necessary. The most common presentation is with recurrent mouth ulcers, often with genital ulcers; however, it may take some years before diagnostic criteria are met. All patients with idiopathic orogenital ulcers should be kept under review, with periodic focused assessment to detect evolution into Behçet's disease. There is often a delay of several years between patients fulfilling diagnostic criteria and a diagnosis being made, which may contribute to the morbidity of this condition. Despite considerable research effort, the aetiology and pathogenesis of this condition remains enigmatic.

Update on the therapy of Behçet disease

“…Topical treatment with CS, topical anesthetics, antimicrobial mouthwashes, sucralfate [Keogan, 2009] and topical granulocyte colony-stimulating factor [Bacanli et al 2006] can be helpful in the management of mucocutanous lesions. Additionally, a number of antibiotics and antivirals, based on the possible role of herpes and Streptococcus sanguinis in the pathogenesis of the disease, have been used primarily for the treatment of mucocutaneous manifestations with variable responses [Calguneri et al 1996a[Calguneri et al , 1996bDavies et al 1988] and thus are not part of the routine care of patients with BD.…”

Use of colposcopy in a patient with recurrent genital ulcers

“…It is a multisystem vasculitic disorder and the diagnosis is based on clinical criteria. [1][2][3][4] Our patient fulfilled the criteria of recurrent oral and genital ulcerations as well as a cutaneous manifestation.…”

A 29‐year‐old woman with headache, fever, right leg numbness, and dysphagia

“…These lesions, which are typically painful but occasionally asymptomatic, particularly in women, typically appear on the scrotum of male patients and on the labia of female patients (18). In addition, the patient has also had none of the other protean potential complications of Behçet's syndrome, particularly cutaneous disease, which can include erythema nodosum, a papulopustular rash, superficial thrombophlebitis, and the pathergy phenomenon (20).…”

“…Unfortunately, the long-term clinical outcomes of patients treated in this manner were often poor, and the treatment regimens themselves were associated with significant morbidity (18).…”