The German

In order to meet this need, the charity German Registry of Adamantiades-Behget's Disease5 and additionally a German net of dermatological practice offices were established; the latter are familiar with the various faces of ABD.

With their help, patients are led in their struggle through the right diagnostic procedures in cooperation with other specialists who are also instructed by the dermatologists.

Dermatologists take a leading position in ABD. In contrast to other countries, dermatological symptoms like aphthous ulcers, genital ulcers and other skin disorders are the first symptoms in Germany and by far more frequent than other disorders. Eye diseases are less common than in Japan for instance, the same is true for arthritis. In addition dermatologists are available throughout Germany, in short distances and without waiting time.

More than 3500 dermatologists in private practices and more than 500 in hospitals provide dermatological health care. Direct access to dermatologists for all patients is granted, no matter how much a patient earns or which health insurance covers the costs. This is different from health care systems like, for instance, in the UK.

A study on the reputation of dermatology in spring 2002, when 1000 people of all ages and social classes were interviewed, showed that dermatologists enjoy a high reputation for all skin related disorders.77% would immediately see a dermatologist for these symptoms. In 19% they first consult the general practitioner, who usually refers ABD patients to dermatologists, at least when the first symptoms reoccur or when patients do not find relief by symptomatic treatment that was offered.There are 5 steps providing appropriate service for ABD-patients. Step 1 is a basic diagnosis at first consultation which can be offered by any dermatologist and by those of other specialties who are familiar with ABD. This means it must be checked whether there is a main symptom like oral aphthous ulcers (min. 3x/year; Fig. 1) plus 2 ofthe 4 following criteria:

- Genital ulcers

- Uveitis (iritis, retinitis)

- Skin disorders (erythema nodosum, folliculitis, sterile pustules)

- Pathergy test positive based on the diagnostic criteria of the „International Study Group for Behcet's disease".

If ABD is considered at step 2 physicians may refer patients to one of the approx. 25 dermatological centers for ABD, now built up on a private initiative throughout the whole country. There, blood test and extensive clinical examination can be performed (Table 2). The patient is referred to a number of specialists who cooperate in a 3rd step when results are compiled and finally offer an appropriate treatment as step 4.

Table 2. Laboratory examinations to be advised in Adamantiades-Behcet's disease

Laboratory parameters for differential diagnostic purposes

Disease-associated laboratory parameters

Blood count

Anticardiolipin antibodies

Differential blood count

HLA-Typing

Iron

c-reactive protein

Vit.B12/Ferritin

Erythrocyte sedimentation rate (ESR)

Transferrin

Rheumatic factor

Complement C3,C4

Antinuclear antibodies (ANA)

Liver tests

Creatinine

Antistreptolysin titer

Yersinia serological tests

Herpes simplex virus - Ak

Coxsackie - Ak

Echoverus - Ak

Hepatitits - Serological tests

Figure 1. Characteristic aphthous ulcers at the mucosa of the lower lip

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