This Behcet’s disease syndrome criteria calculator assesses the positive or negative diagnosis of Behcet’s syndrome based on major and minor criteria. Below the form there is information on each of the findings in the criteria, description of some of the signs and more guidelines on this inflammatory disease.
How does this Behcet’s disease syndrome criteria calculator work?
This health tool helps the diagnosis of Behcet’s syndrome based on clinical and physical examination findings.
The Behcet’s disease syndrome criteria calculator provides the major and minor criteria used in the diagnosis and upon selection of those that apply, is able to give an indication on whether the result is positive or negative.
The major criteria which is compulsory for positive diagnosis is: Recurrent oral ulcerations that can be characterized by minor aphthous, major aphtous or herpetiform ulceration which recurred at least 3 times in one 12 month period:
■ Minor aphthous ulcerations – painful sores that are pale yellow in color but the area around is swollen and red. They usually develop one at a time and last between a week and two weeks without any scarring.
■ Herpetiform ulcerations – are less common than aphthous ulcerations, are pinhead size and may occur more at the same time. They are not related with the herpes virus infection.
The minor criteria provides four examples of findings, from which a minimum of two must accompany the major criteria in the positive diagnosis:
■ Recurrent genital ulceration: aphthous ulceration or scarring;
■ Eye lesion: anterior uveitis, posterior uveitis, or cells in vitreous on slit lamp examination or retinal vasculitis evidenced by ophthalmologist;
■ Skin lesions: erythema nodosum, pseudofolliculitis or papulopustular lesions, or acneiform nodules (observed during examination in post-adolescent patients not on corticosteroid treatment);
■ Positive pathergy test (Behcetine test) read by physician 24-48 hours. The pathergy test consist of pricking the skin of the forearm with a sterile needle. If a red nodule or pustule larger than 2 mm in dimension develops in 24 to 48 hours, then the test is positive.
Some of the terms used above are explained:
■ Anterior / Posterior uveitis – this is the inflammation of the eye tissue provoking swelling and destruction. It is not limited to the uvea and can affect the retina and the optic nerve, thus limiting vision;
■ Retinal vasculitis – is the inflammation of the vascular branches of the retinal artery met in other conditions such as sarcoidosis or multiple sclerosis;
■ Erythema nodosum – this is the inflammation of the fat cells under the skin, manifesting as tender and red nodules or lumps. It usually affects the shin area and resolves by itself in about a month;
■ Pseudofolliculitis – defines ingrown curved hairs that provoke foreign body reaction and inflammation, usually in the area of the beard in men;
■ Acneiform nodules – form of the lesions in acneiform eruption.
Behcet’s syndrome medical guidelines
This is an inflammatory condition affecting several parts of the body at the same time. It affects the lining of the mouth or genital areas and the skin. It also causes joint pain, stomach disorders and fatigue.
Other organs that may be affected include the eyes, blood vessels and nerves.
The cause is yet unknown but the general consensus is that of genetic predisposition, despite the fact that the condition is usually obtained and not directly present at birth.
The symptoms differ from patient to patient and their severity may increase in time if left unadressed:
■ Mouth symptoms – include painful sores similar to canker sores that heal in up to three weeks but recur;
■ Skin symptoms – vary from acne like sores to red nodules, especially localized on the limbs;
■ Genital symptoms – red, inflamed sores on the scrotum or vulva;
■ Joint symptoms – swelling and pain, usually located in the knees, but ankles and wrists may also be affected;
■ Eye symptoms – temporary but recurrent uveitis that causes redness, pain and blurred vision;
■ Blood vessel symptoms – inflammation of vessels that leads to redness, swelling and blood clots;
■ Stomach symptoms – vary from abdominal pain, bleeding, diarrhea.
Diagnosis comprises of physical examination and evaluation of symptoms and diagnostic tests such as lesion biopsy (oral, skin or genital), blood tests for inflammation (ESR, CRP), lumbar puncture or MRI. The diagnosis for this condition is a lengthy process, often taking months or years in a row.
Current treatment protocol is aimed at reducing inflammation and addressing localized symptoms.
Some examples include high dose corticosteroid therapy, anti TFN therapy for uveitis, interferon alpha-2a for oral and genital ulcers and colchicines for erythema nodosum.
1) International Study Group for Behçet's Disease. (1990) Criteria for diagnosis of Behçet's disease. Lancet; 335(8697):1078-80.
2) Davatchi F. (2012) Diagnosis/Classification Criteria for Behcet’s Disease. Patholog Res Int; 2012: 607921.
3) Yazici H, Yazici Y. (2014) Criteria for Behçet's disease with reflections on all disease criteria. J Autoimmun; 48-49:104-7.
4) Suzuki Kurokawa M, Suzuki N. (2004) Behcet’s disease. Clin Exp Med; 4(1):10-20.15 May, 2016