A 27-year-old woman presented with several days of profound fatigue, fever, headache, cervical lymphadenopathy, mouth and genital ulcers.

BEHCET'S SYNDROME

Behçet's syndrome (BS), Behçet's diseased (BD), Behçet syndrome and Behçet disease are the same condition, also known as simply Behçet's. It is an inflammatory condition whose most characteristic features are recurrent oral and genital ulcerations. Ocular, arthritic, neurologic, vascular, GI, and pulmonary lesions may occur as well.

Peak incidence is from 20-40 years of age.
In the United States, the disease is more common in females (5:1 female-to-male ratio), while in the Middle East, males are more affected (e.g., 3.8:1 in Israel, 5.3:1 in Egypt).
One study found a higher incidence of cancer in patients with BS, especially leukemia, lymphoma and oral and pharyngeal cancers.
Mouth and genital ulcers with inflamed cartilage (MAGIC syndrome) is a very rare disease in which the patient fulfills criteria for both Behcet's Syndrome and relapsing polychondritis.
Deep, tender nodules of the shins resembling erythema nodosum are not uncommon.

Clinical

The patient, usually a woman, may present with a variety of signs and symptoms including mouth and genital ulcers, arthritis, eye inflammation, fever, headache etc.

The oral ulcerations resemble apthosis. The genital ulcerations may affect the labia, vaginal introitus, or scrotum. Deep, tender nodules of the shins resembling erythema nodosum are not uncommon. Scattered inflammatory, acneiform papules and pustules may be seen. Uncommon cutaneous manifestations include polyarteritis nodosa, Sweet's syndrome-like lesions, pyoderma gangrenosum-like lesions, EM-like lesions, infiltrated erythema, palpable purpura, hemorrhagic bulla, superficial migratory thrombophlebitis, extragenital ulcerations, and pathergy. Nail fold capillary abnormalities are present in most patients with Behcet's disease.

Revised International Criteria for Behcet's Disease

Sign/Symptom	Score
Oral aphthosis	2
Genital aphthosis	2
Ocular manifestations	2
Skin manifestations	1
Vascular manifestations	1
CNS involvement	1
Positive pathergy test	1

Total of 4 or more is diagnostic for Behcet's syndrome.

Pathergy

The pathergy test may be performed in two ways. First clean the skin of the flexor aspect of the forearm and then:

Prick the skin intradermally with a sterile hypodermic needle (e.g., size 25G).
Inject the skin intradermally with 0.1 ml of 0.9% saline.

Read 48 hours later:

Negative (normal skin)
Positive (red and raised)
Strongly positive (red and raised with a pustule)

Workup

The genital ulcerations should be cultured for herpes. The patient should be seen by an ophthalmologist. Review of systems and physical examination should be complete but with a special focus on cutaneous, gastrointestinal, neurologic, and arthritic symptoms. Appropriate laboratory data include ANA, ASO (Strep may have precipitated the episode), urine analysis, CBC, and G6PD (in case the patient is to receive dapsone).

Cases of Epstein Barr virus can mimic BD, e.g. fever, oral and genital ulcers (Lipschitz). In fact, some cases of EBV infection have "triggered" BD.

Behcet's Syndrome vs. Lipschutz Genital Ulceration

Lipschütz ulcers and Behçet's disease can both cause genital ulcers, but Lipschütz ulcers are a rare, idiopathic condition that typically occurs in young, sexually inactive women, while Behçet's disease is a more systemic autoimmune disorder that can affect multiple areas of the body, including the mouth, genitals, eyes, and skin.

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