Arch Dermatol Res DOI 10.1007/s00403-009-0990-2 123 MINI REVIEW Behçet’s disease: an algorithmic approach to its treatment Erkan Alpsoy · Ayse Akman Received: 30 April 2009 / Revised: 10 July 2009 / Accepted: 5 August 2009 © Springer-Verlag 2009 Abstract Behçet’s disease (BD) is a chronic, relapsing, systemic vasculitis of unknown etiology with the clinical features of mucocutaneous lesions, ocular, vascular, articu- lar, gastrointestinal, urogenital, pulmonary, and neurologic involvement. Mucocutaneous lesions Wgure prominently in the presentation and diagnosis, and may be considered the hallmarks of BD. Therefore, their recognition may permit earlier diagnosis and treatment. Although, the treatment has become much more eVective in recent years, BD is still associated with severe morbidity and considerable mortal- ity. The main aim of the treatment should be the prevention of irreversible organ damage. Therefore, close monitoring, early and appropriate treatment is mandatory to reduce morbidity and mortality. We reviewed the current state of knowledge regarding the therapeutic approaches for BD and designed a stepwise, symptom-based, algorithmic approach, mainly based on controlled studies and our clini- cal experience in this Weld to provide a rational framework for selecting the appropriate therapy along the various treat- ment choices. Keywords Behçet’s disease · Treatment · Algorithmic approach · Mucocutaneous symptoms · Ocular symptoms · Articular symptoms · Gastrointestinal symptoms · Neurologic symptoms Introduction Behçet’s disease (BD) is a chronic, relapsing, systemic vas- culitis of unknown etiology with the clinical features of mucocutaneous lesions, ocular, vascular, articular, gastro- intestinal, urogenital, pulmonary, and neurologic involve- ment [6]. The disease usually starts around the third decade of life. Recent epidemiologic surveys [16, 29, 63] suggest that sex distribution is roughly equal in BD. The disease is particularly prevalent in “Silk Route” populations but has global distribution. The prevalence of BD in Turkey is found to be nearly 1/250 of the population aged 12 or older [16], whereas that in England is less than 1/100.000 [13]. This marked geographic variation of BD can be explained by the genetic basis of the disease and/or environmental triggers. The diagnosis is based on clinical criteria as there is as yet no pathognomonic test. Although several immuno- logical abnormalities have been demonstrated, the exact mechanism of the inXammatory changes occurring remains to be elucidated. The most probable hypothesis is that of an inXammatory reaction set oV by infectious agents such as herpes simplex virus 1 or streptococcus species or by an autoantigen such as heat schock proteins in genetically pre- disposed individuals [4]. Mucocutaneous lesions Wgure prominently in the presen- tation and diagnosis, and may be considered the hallmarks of BD. Oral ulcers (OU), genital ulcers (GU), and cutane- ous lesions together with ocular lesions and arthropathy are the most frequent features of the disease in all countries. Mucocutaneous lesions often precede other manifestations. Therefore, their recognition may permit earlier diagnosis and treatment, with salutary results [13]. OU and GU of BD are characterized by recurrent and painful ulcerations of the oral mucosa and genital skin/mucosa. Relapsing bipolar OU and GU are strongly evocative of BD. They are identical The study was supported by Akdeniz University ScientiWc Research Projects Unit. E. Alpsoy (&) · A. Akman Department of Dermatology and Venerology, Akdeniz University School of Medicine, Antalya 07070, Turkey e-mail: ealpsoy@akdeniz.edu.tr