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About Behçet's Disease

Manifestations of Behçet's Disease

Behçet’s Disease is a rare, chronic, multisystem inflammatory disease. Behçet’s Disease is a form of variable-vessel vasculitis, affecting both arteries and veins of all sizes. The most common manifestation is mucocutaneous lesions accompanied by lesions in other organ systems.^1-3

Behçet’s disease affects multiple organ systems and presents to multiple different manifestations^2,4,5

OCULAR DISEASE

50%

+

OCULAR DISEASE

50%

Including: Anterior uveitis, relapsing hypopyon, vitritis, retinal infiltrates, retinal vasculitis, retinal vascular occlusion.

Presenting specialist: Ophthalmologist.

View an image of ocular disease here.

VASCULAR

16%

+

VASCULAR

16%

Including: Ischemic heart disease; cardiac, pulmonary, and peripheral vessel aneurysms; deep venous occlusions.

Presenting specialists: Cardiologist, pulmonologist, vascular surgeon.

GASTROINTESTINAL

0%-25%

+

GASTROINTESTINAL

0%-25%

Including: Aphthous ulcers in any part of the GI tract.

Presenting healthcare professionals: Primary care physician, gastroenterologist.

GENITAL ULCERS

80%

+

GENITAL ULCERS

80%

Including: Scrotal lesions, vulval lesions, epididymitis.

Presenting specialists: Urologist, gynecologist.

View images of genital ulcers here.

NEUROLOGICAL

5%-25%

+

NEUROLOGICAL

5%-25%

Including: Parenchymal disease, cerebral sinus thrombosis.

Presenting specialist: Neurologist.

ORAL ULCERS

98%

+

ORAL ULCERS

98%

Including: Aphthous ulcers in the mouth and/or throat.

Presenting healthcare professionals: Primary care physician, dentist, oral surgeon.

View images of oral ulcers here.

SKIN LESIONS

80%

+

SKIN LESIONS

80%

Including: Folliculitis, erythema nodosum, pathergy response.

Presenting specialist: Dermatologist.

View images of skin lesions here.

JOINTS

45%

+

JOINTS

45%

Including: Non-obliterative arthritis, usually in large joints.

Presenting specialist: Rheumatologist.

The manifestations of Behçet’s Disease are recurring and remitting and may not occur simultaneously. They may present as seemingly isolated conditions and are often treated by different medical specialists, depending on the system affected. For example, oral ulcers, the most common onset manifestation, are often treated by a dentist or primary care physician. Similarly, a dermatologist will typically see and treat skin manifestations, while a gynecologist or urologist may treat genital ulcers.⁴

Suspect Behçet’s disease?
Refer to a rheumatologist to confirm a diagnosis

What does Behçet's Disease look like?

Images of Behçet’s disease manifestations

Major-type Oral aphthous ulcer

Major aphthous oral ulcers occur as one to ten very painful ulcers with diameters of 10 to 30 mm. They usually last for up to 6 weeks, and about 64% heal with scarring. They are most often found on the lips, tongue, and buccal mucosa, but they also affect the pharynx, palate, and tonsils.

References: 1. Lee S, Bang D, Lee E-S, Sohn S, eds. Behçet's Disease: A Guide to Its Clinical Understanding. Springer-Verlag; 2001:19-50. 2. Barnes CG. History and diagnosis. In: Yazici Y, Yazici H, eds. Behçet's Syndrome. Springer; 2010:7-33.

hypopyon-panuveitis

Hypopyon Panuveitis

The presence of a hypopyon in the eye is associated with a severe panuveitis in the majority of instances. It is seen in around 6-12% of patients with ocular involvement.

Barnes CG. In: Yazici Y, Yazici H, eds. Behçet's Syndrome. Springer; 2010:7-33.

Scrotal ulcer healing with scarring

90% of large genital ulcers (most commonly found on the scrotum in males) that are ≥1 cm in diameter usually lead to scarring.

Barnes CG. In: Yazici Y, Yazici H, eds. Behçet's Syndrome. Springer; 2010:7-33.

4_scrotalUlcer_0003_4-Scrotal-ulcer

Scrotal Ulcer

In males, 89% of genital ulcers appear on the scrotum. They usually begin as a papule, pustule, or circumscribed necrosis that ulcerates within a short period of time. They are frequently painful, round to oval, punched out in appearance, and usually covered with yellowish fibrin or crust with regular and edematous borders.

Barnes CG. In: Yazici Y, Yazici H, eds. Behçet's Syndrome. Springer; 2010:7-33.

acneiform-skin-lesion

Acneiform Skin Lesion

Acneiform lesions are a frequent skin manifestation of Behçet’s Disease prevalent in 30-96% of patients. They present as inflammatory papules, pustules, nodules, and noninflammatory comedones not atypical from acne vulgaris but frequently involve the extremities in Behçet’s Disease.

Barnes CG. In: Yazici Y, Yazici H, eds. Behçet's Syndrome. Springer; 2010:7-33.

erythema-nodosum

erythema nodosum

Observed in up to 50% of patients, erythema nodosum–like lesions are characterized by the presence of red, tender, erythematous non-ulcerating nodules that are 1-5 cm in diameter and symmetrical in distribution. They are frequently found in the lower extremities such as buttocks, lower part of the thighs, and ankles.

Barnes CG. In: Yazici Y, Yazici H, eds. Behçet's Syndrome. Springer; 2010:7-33.

herpetiform-oral-aphthous-ulcers

herpetiform oral aphthous ulcers

These ulcers are made up of recurrent crops of up to 100 small, painful ulcers measuring 1-2 mm in diameter that can join to form larger lesions. They usually last for 7-10 days and about 32% heal with scarring.

Lee S, Bang D, Lee E-S, Sohn S, eds. Behçet's Disease: A Guide to Its Clinical Understanding. Springer-Verlag; 2001:19-50.

minor-type-oral-aphthous-ulcers

minor-type oral aphthous ulcers

Minor aphthous oral ulcers have a diameter of less than 10 mm and usually appear in clusters of one to five small, moderately painful ulcers which last for 4 to 14 days. Only about 8% of these types of ulcers leave scars.

Lee S, Bang D, Lee E-S, Sohn S, eds. Behçet's Disease: A Guide to Its Clinical Understanding. Springer-Verlag; 2001:19-50.

Frequency of Behçet’s Disease manifestations first observed by a healthcare professional^6,*

Oral Ulcers

100%

Genital Ulcers

95%

Papulopustular Lesions

83%

Erythema Nodosum

62%

Eye Disease

47%

Arthritis

39%

Vascular Disease

21%

Neurologic Disease

3%

Patients (%)

*Study of 428 patients whose first clinical visits were between July 1977 and December 1983 (inclusive) in the multidisciplinary Behçet's Disease outpatient clinic at Cerrahpaşa Medical Faculty of the University of Istanbul, Turkey.⁸

The manifestations of Behçet's Disease vary widely in type and severity, with the potential to impact multiple organ systems. However, nearly every person with Behçet’s has frequent, recurrent oral ulcers, and typically, multiple ulcers at once. These ulcers are painful and can be refractory to treatment. Often the first manifestation of Behçet’s, oral ulcers contribute substantially to diminished quality of life (QoL) as a result of their debilitating impact on daily functions such as chewing, swallowing, and speaking. Recurrent oral ulcers in Behçet’s tend to crop and scar.^7,8

In addition to oral ulcers, many people experience other painful manifestations such as arthritis, genital ulcers, and skin lesions. The pain caused by these manifestations can also negatively impact QoL, with visible manifestations also affecting body image.⁸

QoL, quality of life.

Cause of Behçet's Disease

No single underlying cause of Behçet's Disease has been identified. It is thought that genetic predisposition and environmental triggers may work together to activate an autoimmune cascade that leads to Behçet's Disease.³ The exact mechanism of the inflammatory changes in Behçet's Disease remains unknown.^3,4

Behçet’s Disease: An uncontrolled autoimmune cascade in vascular tissue^9-15

Environmental Triggers

Viral or bacterial agents such as Streptococcus sanguinis, herpes simplex virus, Epstein-Barr virus, and cytomegalovirus
Heat shock proteins
Pollution

Genetic Predisposition

Family history
HLA-B51
IL-10
IL23R/IL12RB2

Immune Response Activation

Increased Production Of Pro-inflammatory Cytokines

IL-2, IL-6, IL-12, IL-17, IL-23, IL-33, TNF-α, IFN-γ

Inflammation

HLA, human leukocyte antigen; IL, interleukin; TNF, tumor necrosis factor; IFN, interferon.

Management of Behçet’s Disease

Currently, there are limited approved therapies available to treat Behçet’s Disease. Symptom management strategies involve anti-inflammatory and immunosuppressive agents. Long-term treatment with some agents may be limited by their side-effect profile.⁴

Due to the involvement of different organ systems, guidelines recommend a multidisciplinary approach to managing the manifestations of Behçet’s Disease. While a rheumatologist may be engaged to manage inflammation and will lead the multidisciplinary team, a specialist in a specific area, such as a dermatologist or dentist, may offer symptom management for skin lesions or oral ulcers, respectively.¹

Help raise awareness of the
challenges of Behçet’s Disease and reduce the delay in diagnosis

The most common manifestations of Behçet’s Disease, mucocutaneous lesions, frequently contribute to misdiagnoses.³ Sharing the information below via social media will help raise awareness of Behçet’s Disease and may even prevent a delay in its diagnosis.

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References:

1. Hatemi G, Christensen R, Bang D, et al. Ann Rheum Dis. 2018;77(6):808-818.

2. Barnes CG. History and diagnosis. In: Yazici Y, Yazici H, eds. Behçet's Syndrome. Springer; 2010:7-33.

3. Leonardo NM, McNeil J. Int J Rheumatol. 2015;2015:945262.

4. Zeidan MJ, Saadoun D, Garrido M, Klatzmann D, Six A, Cacoub P. Auto Immun Highlights. 2016;7(1):4.

5. Treatment. American Behcet's Disease Association website. behcets.com. Accessed October 4, 2018.

6. Kural-Seyahi E, Fresko I, Seyahi N, et al. Medicine (Baltimore). 2003;82(1):60-76.

7. Ambrose NL, Haskard DO. Nat Rev Rheumatol. 2013;9(2):79-89.

8. Canpolat Ö, Yurtsever S. Asian Nurs Res (Korean Soc Nurs Sci). 2011;5(4):229-235.

9. Cho SB, Cho S, Bang D. Yonsei Med J. 2012;53(1):35-42.

10. Galeone M, Colucci R, D'Erme AM, Moretti S, Lotti T. Patholog Res Int. 2012;2012:595380.

11. Hatemi G, Seyahi E, Fresko I, Talarico R, Hamuryudan V. Clin Exp Rheumatol. 2017;35(suppl 108):3-15.

12. Na SY, Park MJ, Park S, Lee ES. Clin Exp Rheumatol. 2013;31(3 suppl 77):32-40.

13. Chi W, Zhu X, Yang P, et al. Invest Ophthalmol Vis Sci. 2008;49(7):3058-3064.

14. Lopalco G, Lucherini OM, Lopalco A, et al. Front Immunol. 2017;8:200.

15. Nanke Y, Yago T, Kotake S. J Clin Med. 2017;6(7).