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Oral. Oral ulcerations represent the most common finding of disease and are seen in over 95% of patients with Adamantiades-Behget's. They are required for diagnosis (Table 1), although some argue that in rare cases, oral ulcers need not be present for the diagnosis to be made. The oral ulcerations seen in Adamantiades-Behget' s may be present on the tongue, lips, gingival surfaces, buccal mucosa, soft palate, or posterior pharynx (Fig. 1). In general, they are shallow and painful, resolving in one to three weeks, usually healing without scarring. The ulcers (<3 mm) can range in size from small, herpetiform lesions

TABLE 1 Clinical Manifestations of Behçet's Disease

Oral ulcers (96-100%)


Genital ulcers (65-90%)

Demyelinating disease

Systemic manifestations

Vasculitic brain stem or cord lesions

Weight loss

Intracranial aneurysms


Venous thrombosis


Peripheral neuropathy


Gastrointestinal manifestations


Mucosal ulcerations

Skin manifestations

Intestinal perforations


Budd-Chiari syndrome

Erythema nodosum

Pulmonary manifestations (8%) (16)

Pathergy test

Thromboembolic disease

Acneiform lesions

Pleural disease

Superficial thrombophlebitis

Bronchiolitis obliterans/organizing

Articular manifestations



Pulmonary hypertension

Arthritis (polyarticular or monoarticular)

Interstitial lung disease/fibrosis

Ocular manifestations (75%) (14)

Pulmonary artery aneurysms


Cardiac manifestations

Anterior uveitis

Conduction abnormalities

Posterior uveitis

Myocardial fibrosis


Vasculitic infarction

Retinal vasculitis


Optic neuritis

Aortic aneurysms

Venous thrombosis


Glaucoma (secondary)

Nailfold capillary abnormalities

Retinal neovascularization


Vitreal bleeding


Neurologic manifestations (20%) (15)

Amyloid deposition


Aseptic meningitis

(<2mm) to larger lesions (>2cm). They can be found in focal areas or diffusely throughout the oropharynx. Oral ulcers may develop or worsen after patients ingest hard substances such as nuts or crackers, likely representing an inflammatory process triggered by microtrauma, similar to the pathergy response described next, although in the case of English walnut ingestion, there may also be an immunomodulatory function of the nuts (18,19). Interestingly, cigarette smoking may decrease the frequency of oral ulcers (20).

Ocular. Ocular disease is common in Adamantiades-Behçet's and in one series, occurred in some form in up to 75% of patients (14). Uveitis, the eye manifestation most commonly seen, is often bilateral and recurrent, and can involve the entire uveal tract, findings that are unusual in other autoimmune diseases of the eye. Severe anterior uveitis may lead to hypopyon, with inflammatory cells accumulating in the anterior chamber and, this finding, assuming that infection is not present, can be very suggestive of Adamantiades-Behçet's disease (Fig. 2) (21). Isolated posterior uveitis, venous vascular occlusion, and optic neuritis may also occur and may rapidly progress to blindness if left untreated (Fig. 3). Other ocular changes seen include secondary cataracts and glaucoma from inflammation and corticosteroid use, adhesions of the iris to the lens, retinal neovascularization with vitreal bleeding, and conjunctival ulceration (Fig. 4). Eye disease usually appears at the same time as other manifestations of Adamantiades-Behçet' s, but in some cases, can present late in the disease course, and subclinical inflammation may persist in between major flares, leading to progressive vision loss. Predictors for more severe vision loss include young age at onset,

FIGURE 1 Oral ulcerations in Behget's disease. Source: Courtesy of Pamela Chavis, MD. From Ref. 17.

male gender, bilateral disease, and panuveitis or retinal vasculitis; these patients should be treated aggressively to prevent blindness (14).

Systemic Manifestations

General Manifestations. Patients with Adamantiades-Behget's can experience any number of nonspecific symptoms including malaise, anorexia and weight loss, generalized weakness, fevers, lymphadenopathy, and headache (10). In some cases, these symptoms can predate the appearance of ulcerations, making initial diagnosis difficult.

Articular. The arthritis seen in Adamantiades-Behget's is variable, with polyarticular and monoarticular forms seen. The most commonly involved joints are the knees, wrists, ankles, and elbows. The arthritis is typically inflammatory but nonerosive. Sacroiliitis is not typically seen with Adamantiades-Behget's and, if present, may represent HLA-B27-associated disease.

Cutaneous. There are multiple skin lesions described in Adamantiades-Behget's, including erythema nodosum, papular/pustular eruptions, folliculitis, and lesions similar to acne (Figs 4 and 5). The classic lesion is termed "pathergy," where a pustular lesion develops at a site of recent trauma (Figs 6 and 7). In the clinical setting, these lesions can occur at the site of needle punctures, and for diagnosis, are defined as the occurrence of a papular/pustular lesion at the site of oblique angle needle stick (20-25 gauge) 24 to 48 hours

FIGURE 2 Anterior uveitis with hypopyon in Behget's disease. Source: Courtesy of Pamela Chavis, MD. From Ref. 17.

FIGURE 3 Retinal vasculitis with blurred disk margins and vascular abnormalities. Source: Courtesy of Pamela Chavis, MD. From Ref. 17.

later. Recurrent genital ulcerations also occur, with features similar to the oral ulcerations described above, although they tend to scar more frequently than the oral lesions (Fig. 8). Genital ulcerations can be located externally on the vulva, scrotal or penile tissue, or internally in the vaginal vault and on the cervix. They can also appear in the groin region and can rarely occur on the perineum and perianal area. A subset of Adamantiades-Behget's that includes mouth and genital ulcers with inflamed cartilage has been termed the "MAGIC" syndrome and likely represents an overlap between Adamantiades-Behget's and relapsing polychondritis (21).

Nervous System. Neurologic involvement in Adamantiades-Behget's can be seen in up to 20% of patients (15). Multiple nervous system lesions have been described, including brain parenchymal disease, vasculitic brain stem or spinal tract involvement, aseptic meningitis, venous thrombosis, aneurysms, and peripheral neuropathy. Central nervous system involvement usually indicates a poorer prognosis, especially if abnormal cerebrospinal fluid is found or brain parenchymal disease is present (23).

Gastrointestinal. Patients with Adamantiades-Behget's may have upper gastrointestinal (GI), gastric, small bowel, or large bowel involvement. GI ulcerations are similar in appearance to those seen in the orogenital regions and can lead to significant bleeding or intestinal perforation (4,5). If GI ulceration is present, evaluation for inflammatory bowel disease such as Crohn's disease or ulcerative colitis, both of which can mimic Adamantiades-Behget's, should be considered.

Pulmonary. Lung manifestations may be present in up to 8% of patients with Adamantiades-Behget's; these include thromboembolic events, inflammatory lung disease with pleuritis, bronchiolitis obliterans, vasculitic pneumonias, pulmonary hypertension, and

FIGURE 4 Cataract and posterior synechiae in Behget's disease. Source: Courtesy of Pamela Chavis, MD. From Ref. 17.
FIGURE 5 Skin lesions in Behget's disease; (A) Ulcerative lesion; (B) pustular lesion; (C) Acneiform/pustular lesions; (D) erythema nodosum. Source: Courtesy of Pamela Chavis, MD. From Ref. 17.
FIGURE 6 Mild pathergy in Behget's disease. Source: Courtesy of Pamela Chavis, MD. From Ref. 17.

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