Elsevier

Clinics in Dermatology

Volume 17, Issue 2, March–April 1999, Pages 209-223
Clinics in Dermatology

Reviews
Behçet’s disease in the Middle East

https://doi.org/10.1016/S0738-081X(99)00013-9Get rights and content

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History

Prof. Dr. Hulusi Behçet was a specialist of dermatology and venereology who lived from 1889 to 1948, and was the founder of the Dermatology and Venereology Clinic of Istanbul University. He first saw a patient in 1924/25 who had oral and genital aphthae, eye involvement, and nodular lesions in his legs and who was followed up for 40 years by doctors from Vienna and Istanbul.

This patient was followed with the diagnoses of “aphte recidivante chronique” and “erythema exudativum multiformis”

Epidemiology

The prevalence of BD is high in Japan, China, Korea, Turkey, Iran, Tunisia, and in the Mediterranean and Middle Eastern countries whereas it is low in Northern Europe and in the United States. The frequency of the disease seems to follow the ancient Silk Route. The first studies concerning the epidemiology of BD were done in Japan.8 The prevalence of BD in Japan in a hospital-based survey done in 1972 was 7–8.5/100000. The disease seemed to be more frequent in northern Japan compared to the

Genetics

There is no specific mode of Mendelian transmission in BD.21 Some familial cases have been reported,22, 23, 24, 25, 26, 27 but an overall increase in the prevalence of disease in families does not exist. A pair of monozygotic twins concordant and two pairs of monozygotic twins discordant for the disease have been reported.28, 29 The relationship between the histocompatibility antigen HLA-B5 and BD has first been reported by Ohno in Japan.30 It was determined that HLA-B5 produced a relative risk

Pathogenesis

The pathogenesis of Behçet’s disease is not known. There is evidence that the disease is caused by inflammation related to disorders in humoral and cellular immunity in patients who have an appropriate genetic background, but the details are not clear.

Clinical findings

The clinical findings of BD can be grouped into mucocutaneous, musculoskeletal, ophthalmological, vascular, and central nervous system manifestations. Remissions and exacerbations characterize the course of the disease.

Juvenile behçets disease

Patients who fulfill the diagnostic criteria before 16 years of age are categorized as juvenile BD. Lang et al.,98 who had observed a case of juvenile BD, have searched the literature between 1965 and 1990 for pediatric onset BD and have found 36 additional cases. The clinical and demographic characteristics of juvenile onset BD patients in Tunisia, France, Iran, Korea, Turkey, Israel, and Morocco have been analyzed and documented.99, 100, 101, 102, 103 Juvenile BD patients constitute

Histopathology

BD is a vasculitic syndrome. Most of the cutaneous findings consist of neutrophilic infiltration and in some cases of leukocytoclasis and a perivascular mononuclear inflammation.

The histological examination of aphthous and genital ulcerations consist of loss of epidermal tissue and fibrin deposition at the base of the ulcer. Leukocytoclasis in addition to neutrophilic infiltration is observed in early lesions. An inflammatory cellular infiltrate consisting of neutrophils and mononuclear cells

Laboratory findings

The laboratory findings of BD are nonspecific. A mild anemia and leukocytosis is observed in 15% of chronic patients.116 These findings do not correlate with clinical activity. Erythrocyte sedimentation rate and C-reactive protein may slightly increase but they are not markers of disease activity.112

Serum immunoglobulins and especially IgA may increase but rheumatoid factors and antinuclear antibodies are absent. Anti-neutrophil cytoplasmic antibodies (ANCA) and antiphospholipid antibodies113

Differential diagnosis

BD is diagnosed from clinical findings. There are various diagnostic criteria. The most frequently used are the International Study Group59 and Japanese criteria.9 The Middle Eastern countries usually utilize the International Study Group criteria. Ghayed17 in Lebanon uses the Japanese criteria, whereas Davatchi114 in Iran utilizes the modified International Study Group Criteria. The most frequently used set of diagnostic criteria in Turkey consist also of the International Study Group

Therapy

Therapy is planned according to symptoms. Immunosuppressive therapy is very important in young patients who tend to have a more severe course and who have eye disease. Therapy should be started early, before any structural damages in the eyes take place. Azathioprine is an immunosuppressive that has proven to be of benefit in the uveitis of BD in a double blind controlled study.117 It is utilized in a dose of 2.5 mg/kg/day and the patients need to be monitored on the basis of bone marrow and

Acknowledgements

The following made contributions to this study: Abdallah MA, Department of Dermatology, Faculty of Medicine, Ain Shams University, Cairo, Egypt; Al Aboosi MA, Department of Dermatology, Jordan University of Science and Technology, Irbid, Jordan; Al Dalaan A, ABIM, FACR, Deputy Director, King Faisal Specialist Hospital and Research Center, Saudi Arabia; Davatchi F, Prof. of Medicine, Head of Division Rheumatology, Tehran University for Medical Sciences, Tehran, Iran; Ghayad E MD, Hotel Dieu De

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