Bhecet’s disease

Bhecet’s (pronounced betchet’s) syndrome was first recognize in Turkey and was originally thougt to be a disease of Mediterrancan origin. However, alarge number of cases have since been reported in Japan and other countries. Women are more commonly affected than men. Bhecet’s syndrome is a rare disease characterized by a clasical triad of RAS (any of the three clinical variants), genital ulceration, and inflamatory eye lesion. Other manifestation include skin, join, neurological, vascular, and intestinal disordes. Up to 90 per cent of affected patiens have RAS. Genital ulcer are smilar to those of the oral mucosa and healing may take plae wich scar formation and lead toresidual tissue loss and deformity smlar to that in the oral cavity. The ocular involvment initially takes the from of anterior uveitis, a superficial inflamatory lesion of the anterior part of the eye, which may become more servere in later episodes and, perhaps, progres to involve other structures of the eye. This may lead to parmanent damage by scar formation or, even, to blindness.

The scquence of involvement of the oral an genital mucosa is variabel, and the two may not involved simulataneously. Indeed, rhere may be a considerabel innterval between the involvement. Ocular involvement, however, is usually late, ocuring sometimes after many years of intermittent oral and genital ulceration. In the generalized from of the disease, skin lesion, of various kind mny appear, themost characteristic being papules that procced tobeing papules that procced to pustule formation. Is the interesting to note that in some patients with skin lesions there is a marked skin resction to trauma. The tendency of sterile blisters to develop at venepuncture sites is kow as ‘pathergy’ , and it may be useful as a siagnostic indicator. Pathergy is less commonly reprtted in UK patients with Bechet’s disease.

The neurological disease that may occure in these patients is the result of the appearance of centers of inflamation and necrosis within the central nervous system. The symptoms are variabel, depending upon the location of the lesion, but in the early stages they may resemble those of multipel sclerosis. There is a vasculitis, perhaps complicated by thrombotic episodes, that may be either localized and minor or involve major vessels. The effect on the joint is that of non-specific arthropathy. It has been suggested that major and minor criteria should be used to arrive at a diagnosis of Bechet’s disease. The major criteria are oral uleration, general ilceration,eye lesion, and skin lesion. The minor criteria ainculde lesion of the nervous system, vascular system, joint, gastrointestinal tract, and pulmonary system. However, there is no agreement as to the number of type of lesion necessary to arrive at the diagnose. An international working party has redefined the criteria for the diagnosis of Bechet’s as the presence of RAS plus any two of : recurrent genital ulceration, eye lesions, skin lesions or positive pathergy test. It is the authors viewpont that, as this a progresive condition, often begining as RAS whitout any other system involvment, it is impossible at any given time to differentiate eith any degree of accuracy between uncomplicated RAS and RAS that might eventually proceed to orogenital ulceration or Bechet’s disease.

Management

The managemnt of Bechet’s syndrome usually requires a multidisciplinary apporoach. However , the local management of oral aphthae in Bechet’s syndrome is exctacly that for all other forms of RAS and is smilarly limited in effect. Systematic therapy is therefore required in most cases

and drugs used include: systemic steroid, azathioprine, cyclophospahamide, cochicine, ciclosporin, and more recentl, anti-TNFα therapy and mycophenolate. Thalidomide appears to be succesful in some cases eith muocitaneous involvment but its use is restricted because of its teratogenicity and side- effects (see above).

Discussion of problem cases

Case 5.1 Discussion

Q1 How would you manage this gentelman and what therapeutic option are avaialabel?

The history and clinical examination of the ulcers in this case consistent with major RAS. It is important specifically to enquire about genital ulceration and other symptoms, ehich might be suggestive of Bechet’s disease. The short history of ulceration is, however, suggestie of a recent precipitating cause of factor, which should be sought. It is important to establish whether the patients stopped smokeing after his myocardial infarction and then devolped RAS, as smoking cessation can precipitate RAS in some individuals. Oral ulceration has been reported in patients on nicocardil, a potassium-channel activator that is used for unstabel angina, and it is important to rule this out as causes of this patient’s oral ulceration. (This must not be stopped before liaising wuth the cardiologist.) Blood test should be arranged to check for raised inflamatory markers, heamitinic deficiencies, and anti-endomysial aoutoantibodies. A full biochemical and immunological profile is also advisabel.

In view of the serverty of RAS and this patiet’s poor quality of life, some form of systemic therapy needs to be considered and the options include system steroids, azathioprine, ciclosporin, thalidomide, and clochicine. In the case of a young patient with a history of coronary artery disease, long-term prednisolone is cintraindicated although a short, reducting course of prednisolone may give short-term relife of ulceration. RAS does, however, eventually recur after stoppingthe steroid. Topical analgesics may be required and an antifungal should also be consider, particulary if there is a high oral carriage rate of candida albicans.

Colchicine has been reported successfu therapy for RAS and azathioprine may be worht considering, either alone or as a steroid-sparing agent. Another treatmen option for this male patient is thalidomide, although this is associated with a number of significant side-effect and the patient will need counselling and close monitoring.

Q2 How would you manage this dental emergecy?

Ther are a number of factors to be considered when arraging extraction of this man’s tooth, including his medical and drug history (previous myocardinal infraction, aspirin) and anxiety about dental treatment. He is probably best treated under local abesthesia, with sedation if required. The aspirin may predispose to postextraction haemorrhage but this is unlikely to be significant and there is no indicaion to stop the medication. It is important to be check for haemostasis after the extraction and, if necessary, suture and/ or pack the socket. If the patient devalops agina then he

should use his glyceryl trinitrate spray sublingually, if there are more serve cardiac complications, then the patient must be treated accordingly.

Cae 5.2 Discussion

Q1 How would you manage the patien in your practice?

Although the patient appears fit and well, she should be questioned about any gut, eye, or skin problems and asked if she has suffered from ulceration. Her doctor can arrange for blood tests but it is important to diplomatically piont out that these should ibclude estimation of ferritin, folate, and B12 levels, as a full blood count and film are insusfficient. (It is important to establish a good working relationship with general medical practitioner in your area)

If this patient’s clinical examination is consistent with a diagnosis of MiRAS and thee is no inclination of sytemic disease, then there are number of treatment option availabel that the dentist can prescribe. Analgesic rinses can be helpful, particularly before meals, and an antiseptic rinse will reduce secondary infection and aid plaque control, particulary if toothbrushing is painful. Hydrocortisone pelletes can be used topicaly in the prodormal phase of ulceration and many resolution of the ulcer. Triamcinolone paste is dificult to apply but may be useful, if applied last thing at night. Any obivious caouse of mechanical truma due o broken teeth or dental application should be eliminated, in case these are precipitating the aphtouse ulcers. More potent topical steroid, in the form of rinses o inhalers may be reqired. In most cases, these simpel measures can reduce the discomfort and duration of RAS. It does, however, need to be pointed out to this patient that there is not, at the prestent, any satisfactory ‘cure’ for this conditions and the risks of systemic therapy probably our weight the benefits in her particular case.

Project

1. Find out whih systemic druhs have been resported as causing oral ulceration, inclucing RAS.