Background
Definition- classification of BD
Recurrent oral aphtosis (mandatory, seen by a physician and recurrence at least 3 times in a year) | |
plus at least two of
| |
Genital ulceration (or aphthosis, at least once)
| |
Skin lesions (erythema nodosum, necrotic folliculitis and papulopustular lesions, (post adolescent, not receiving steroids)
| |
Ocular lesions (posterior uveitis, total uveitis, retinal vasculitis)
| |
Positive pathergy test (done by needle prick (oblique insertion of 20- gauge needle) or intradermal saline injection and read at 48h by a physician. Positive reactions include: papula, pustula or papula with surrounding erythema)
|
Clinical manifestations | Description | Value/item |
---|---|---|
Oral aphthosis | 2 | |
Genital ulceration | 2 | |
Ocular signs | Anterior uveitis, posterior uveitis, retinal vasculitis | 2 |
Skin lesions | Pseudo folliculitis, skin aphthosis, erythema nodosum | 1 |
Neurological manifestation | 1 | |
Vascular signs | Arterial thrombosis, Large vein thrombosis, phlebitis or superficial phlebitis | 1 |
Positive pathergy test
|
(additional criteria)
| 1 |
Item | Description | Value/item |
---|---|---|
Recurrent oral aphtosis | At least 3 attacks/year | 1 |
Genital ulceration | Typically with scar | 1 |
Skin involvement | Necrotic folliculitis, acneiform lesions, erythema nodosum | 1 |
Ocular involvement | Anterior or posterior uveitis, retinal vasculitis | 1 |
Neurological signs | With the exception of isolated headaches | 1 |
Vascular signs | Venous thrombosis, arterial thrombosis, arterial aneurysm | 1 |
Etiology/pathogenesis
Immunology
Genetics
Epidemiology
Clinical symptoms
Fever
Mucocutaneous lesions
Paediatric series | Adult series | ||||||
---|---|---|---|---|---|---|---|
Clinical features in % | Karincaoglu [4] | Krause [37] | Koné- Paut [7] | Atmaca [35] | Koné-Paut + [13] | Alpsoy E [38] | Krause [37] |
N = patients | 33 | 19 | 65 | 110 | 156 | 661 | 34 |
Oral aphthosis | 100 | 100 | 96 | 100 | 100 | 100 | 100 |
Genital aphthosis | 82 | 31.6 | 70 | 83 | 55 | 58.3 | 88.2 |
Cutaneous signs | 89.5 | 92 | 76 | 82.4 | |||
Erythema nodosum | 52 | 36.8 | 40 | 37 | 22.7 | 44.2 | 26.5 |
Pseudo folliculitis | 51 | 8/19 | 58 | 39 | 43 | 55.4 | ND |
Pathergy test + | 37 | 41.2 | - | 45.5 | NS | 37.8 | 57.1 |
Ocular signs | 35b
| 60 | 45 | 29.2 |
Approx 50 % | ||
Anterior uveitis | 47.4 | 31 | 30.1 | 47.1 | |||
Posterior uveitis | 42.1 | 36 | 9 % | 28 | 8.8 | ||
Retinal vasculitis | 10.5 | 16.7 | |||||
Arthralgia/arthritis | 40 | 47.4 | 56 | 22 | 41 | 33.4 | 17.6 |
Gastro intestinal | 4.8 | 36.8 | 14 | NS | 4.49 | 1.6 | 11.7 |
Neurologicala
| 7.2a
| 26.3 | 15 | 3.6a
| 5a
| 3a
| 5.8a
|
Vascular | 9.6 | 10.5 | 15 | 3.6 | 15.38 | 4.4 | 26.5 |
Family history of BD | 19 | ND | 15 | 12 | 24.4 | ND | ND |
Age first symptom (years) | 12.3 (1–16) | 6.9 ± 3.9 | 8.4 (0–16) | 11.6 (1–16) | 7.83 ± 4.39 | ND | 30.4 ± 8 |