BEHCET'S SYNDROME

By Gary M. White, MD

Behcet's Syndrome: ulcer on the labia


Behcet's syndrome (BS) 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.

Clinical

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 [BJD 1999;140;358], 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 [J Rheumatol 1995;22;1108].

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:

Read 48 hours later:

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).

Treatment

As BS is a systemic illness, multiple specialties are often required, with particular emphasis on the ophthalmologist, gastroenterologist, rheumatologist, neurologist, and dermatologist.

Steroids

For urgent, severe, or life-threatening symptomatology (e.g., neurologic, vascular, gastrointestinal, ocular), pulse dose steroids (e.g., 1 g intravenous methylprednisolone infusions daily) are often used for 3 days, followed by 1 mg/kg/day prednisolone tapered slowly.

Infliximab

Skin involvement may be quite bothersome, but does not create the urgency that say ocular involvement might with the potential for permanent visual impairment. In that regard, the combination of infliximab with azathioprine or cyclosporine A or methotrexate appears to be superior to infliximab monotherapy for sustained ocular remission [Semin Arthritis Rheum 2011;41: 61–70]. Infliximab has also been shown effective for most manifestations of BS, including neuro, GI, and mucocutaneous symptoms. It is often given if initial steroid treatment fails.

Etanercept

Etanercept has been shown to reduce the mucocutaneous and arthritic symptoms in 20 patients given 25 mg twice a week [J Rheumatol. 2005 Jan; 32(1):98-105].

Adalimumab

Adalimumab has been shown via multiple small studies or case reports to help with the mucocutaneous side effects as well as ocular, neuro, and vasoocclusive manifestations of BS.

Rituximab

Rituximab has shown some promise in treating ocular as well as other manifestations of BS.

Anakinra

Anakinra 100 mg/day has been reported effective [Ann Intern Med. 2008 Aug 19; 149(4):284-6]. Within 2 weeks, there was dramatic improvement in symptomatology.

Colchicine

Colchicine in several large studies has shown benefit in reducing or preventing skin lesions, vaginal ulcerations, and joint symptoms. Efficacy with regard to preventing oral ulcerations has been less consistent.

Azathioprine

Azathioprine has shown benefit in treating ocular symptoms.

Apremilast

Apremilast 30 mg BID has been shown effective in reducing or eliminating the oral and genital ulcers of BS [N Engl J Med. 2015 Apr 16;372(16):1510-8].

Interferon alfa

Interferon alfa 2a was helpful for the mucocutaneous side effects of Behcet's [AD 2002;138;467].

Thalidomide

Thalidomide 100 mg/day appears effective in treating the orogenital ulcers. Worsening of erythema nodosum lesions as well as a polyneuropathy are potential side effects however.

Dapsone

Dapsone 100 mg/day was superior to placebo in reducing oral/genital ulcers [J Dermatol. 2002 May; 29(5):267-79].

Cyclosporin

Cyclosporin has been used effectively, but increased neurotoxicity has been documented in patients with BS.

Other Therapies

For complex aphthosis or the oral manifestations of BS, many patients are controlled with topical clobetasol (e.g. gel 5-10 times per day).

NSAIDs are often given for the arthritis.

Erythema nodosum lesions have responded to intramuscular Kenalog given every 3 weeks.

Additional Pictures

Multiple ulcerations and maceration in a young woman with Behcet's syndrome. She ultimately was well controlled on infliximab.
Behcet's Syndrome Behcet's Syndrome--multiple ulcers

Behcet's Syndrome: ulcer on the labia

Courtesy Erkan Alpsoy, MD
Behcet's Syndrome of the scrotum

References

PG complicating Behcet's. Indian J Dermatol 2015;60:105

A dramatic case of Behcet's disease successfully treated with infliximab. Dermatology Online Journal 19 (4)

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