Sexually Transmitted Diseases - I. I. Mavrov 2005

Non-Venereal Inflammatory Diseases of the Genital Organs
Behçet's Disease

A systemic disease of unknown Etiology. The Role of viral infection has not yet been proven. According to several authors, Behçet's disease represents an immune-complex vasculitis with predominant involvement of the Venous system.

In 1937, the Turkish dermatologist Behçet described the characteristic triad of the disease: recurrent oral aphthae, genital aphthae, and ocular lesions. With further study, the clinical picture was expanded to include other symptoms caused by the involvement of various Organs and Tissues. Today, Behçet's disease is considered synonymous with mucous membrane aphthosis of the Mouth, genitals, eyes, and Skin, also known as oculoturogenital syndrome.

Clinical Features. As a rule, the initial symptom of the disease is aphthous stomatitis, which occurs in virtually all patients and is characterized by an acute onset and a recurrent course. Aphthae (frequently multiple) are localized on the mucous membrane of the Cheeks, Lips, Tongue, and Pharynx, and are consistently very painful (see insert XV).

The character of aphthous lesions on the external genitalia (present in 79.4% of patients) is similar, and healing in some cases results in scar formation. In men, the sores are painful and located on the Scrotum, prepuce, and glans Penis; in women, they occur in the Vagina or on the cervix and are typically mildly painful.

Ocular involvement (observed in 76% of patients) is most characteristically manifested by The Development of iridocyclitis with hypopyon, anterior and posterior uveitis, chorioretinitis, and vitreous hemorrhages, frequently resulting in Vision loss due to secondary glaucoma, optic atrophy, or cataract.

Skin changes resembling erythema nodosum or erythema multiforme (leaving behind pigmentation) and papulopustular folliculitis are detected relatively frequently (in 60% of patients).

The joints (arthralgia, Arthritis in 60% of patients), Blood Vessels of various localizations (migratory thrombophlebitis), the Central Nervous system (recurrent meningoencephalitis, Spinal Cord lesions, psychiatric disorders), and less frequently, Internal Organs, are also drawn into the pathological process.

Gastrointestinal manifestations (ulcerative esophagitis, enteritis, colitis), as well as involvement of The Heart, Lungs, and Kidneys, are possible, which are presumed to be vascular in origin.

The disease follows a chronic, progressive course with frequent relapses, although relative stabilization of the process may occur in individual organs and systems. Mortality (3–4% of patients) is most often associated with ruptured vascular aneurysms, intestinal perforation, or severe meningoencephalitis.

Diagnosis is based on clinical manifestations, as there are no specific Laboratory tests. According to recent data (European Congress of Rheumatologists, 1987), the major diagnostic Criteria for the disease include: 1) oral mucosal ulceration, 2) genital ulceration, 3) skin lesions (erythema nodosum, pustules), 4) ocular lesions, 5) arthritis, and 6) thrombophlebitis (vasculitis); minor criteria include: 1) neurological symptoms, 2) gastrointestinal lesions, 3) cardiovascular changes, 4) renal involvement, 5) epididymitis, and 6) family history.

Behçet's disease is considered definite in the presence of ulcerative stomatitis combined with three other major criteria; probable in the presence of ulcerative stomatitis and two other major criteria. The combination of ulcerative stomatitis with one other major criterion and two minor criteria defines possible Behçet's disease.

Diagnostic value is also attributed to a positive skin pathergy test with pustule formation resulting from the enhanced leukocyte chemotaxis characteristic of the disease (T. Shimizu et al., 1979). Investigation of the human leukocyte antigen (HLA) system reveals the HLA-B5 antigen in 62–80% of patients, which is considered a genetic marker of the disease (A. Müftüoğlu et al., 1981).

When the characteristic symptom complex is present, diagnosis is relatively straightforward. Difficulties in clinical practice arise due to the fact that not all typical signs appear simultaneously, and they may be preceded by rarer manifestations of the disease (e.g., large vessel involvement, CNS pathology).

Differential diagnosis should be performed with isolated aphthous stomatitis, recurrent oral and genital herpes, Stevens-Johnson syndrome, Reiter's Syndrome, Bekhterew's disease, systemic vasculitis, and others.

Treatment has not yet been clearly established. Opinions in the literature on this issue vary and are sometimes contradictory. Some authors consider the long-term use of immunosuppressants (cyclophosphamide, leukeran) in standard doses to be effective. Others note their effect only on the course of uveitis. High-dose glucocorticoids and, in particular, pulse therapy can temporarily suppress the inflammatory activity of the process and affect systemic manifestations. A combination of corticosteroids and immunosuppressants is possible. There are reports on the efficacy of colchicine, levamisole, antiviral drugs, and plasmapheresis in Behçet's disease. One possible treatment regimen includes: methylprednisolone pulse therapy 1 g intravenously by drip infusion (3 administrations) combined with cyclophosphamide (1 g single dose), plasmapheresis (3 sessions), prednisolone (20 mg/day), tavegil, calcium gluconate, and multivitamins. Local treatment is administered with consideration of eye, skin, and mucosal lesions, among others.