Which skin and mucous findings are characteristic of Behçet disease?

Updated: Dec 21, 2020
  • Author: Nicole Davey-Ranasinghe, MD; Chief Editor: Herbert S Diamond, MD  more...
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Painful oral lesions (aphthous or herpetiform) are one of the criteria for diagnosis and may be the first manifestation (70% of cases). See the image below.

Oral aphthous ulcers secondary to Behçet disease. Oral aphthous ulcers secondary to Behçet disease.

Oral lesions are commonly found in keratinized areas of the oropharynx, often excluding the nonkeratinized surfaces of the dorsal tongue, gums, and hard palate. The lesions are usually not distinguishable from those due to other causes but often have a high recurrence rate (often more than five times per year, despite only three times per year specified in ISG criteria) and appear as multiple lesions or crops (often more than six simultaneous lesions at a given time).

Skin lesions often occur in the genital region of both sexes. In males, scrotal involvement is most characteristic; however, lesions can also develop on the penile shaft. In females, the labial area is most commonly involved, with lesions occasionally developing in the vagina and on the perineum. Genital ulcerations typically heal with scarring and are more painful in men. Development of ulcerations in women may correlate with menstruation.

Nodules that resemble erythema nodosum are more common in the lower extremities of females. They are tender, erythematous, and nodular and usually resolve after 2-3 weeks but often recur. Erythema nodosum may be an indicator of mild Behçet disease.

Acneiform papulopustular lesions are more common in men and are usually found on the trunk and extremities, although they may develop anywhere on the body.

Extragenital ulcerations that heal with scarring are rare and affect only 3% of patients. [33] These are very specific for Behçet disease. They can be found in the axillae, neck, breast, interdigital skin of the feet, and groin.

Positive pathergy test is more common in Turkish and Japanese populations, as well as patients with ophthalmic and neurologic manifestations.

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