A 25-year-old woman comes to the physician because of multiple oral ulcers that have occurred intermittently for the last two months. These ulcers are very painful and are associated with fatigue and low grade fever. She is a teacher and several of her kindergarten students have been ill with hand-foot-and-mouth disease. She has a five-year history of a clinical diagnosis of genital herpes with intermittent flares requiring oral acyclovir. Her mother has an unspecified autoimmune disease with joint pains for which she has taken intermittent steroid therapy over the last 10 years.
Physical examination reveals three vesicular lesions on her buccal and gingival mucosa and acneiform lesions on her face and back. Scars are noted on the patient's vulvar area. The remainder of the physical examination is normal.
Which of the following is the most likely diagnosis?

Question

A 25-year-old woman comes to the physician because of multiple oral ulcers that have occurred intermittently for the last two months.  These ulcers are very painful and are associated with fatigue and low grade fever.  She is a teacher and several of her kindergarten students have been ill with hand-foot-and-mouth disease.  She has a five-year history of a clinical diagnosis of genital herpes with intermittent flares requiring oral acyclovir.  Her mother has an unspecified autoimmune disease with joint pains for which she has taken intermittent steroid therapy over the last 10 years.
Physical examination reveals three vesicular lesions on her buccal and gingival mucosa and acneiform lesions on her face and back.  Scars are noted on the patient's vulvar area.  The remainder of the physical examination is normal.
Which of the following is the most likely diagnosis?

Quizplus · Accepted Answer

11ec193a_991b_1814_853d_4b9cce5bd5e4_MD0002_00 This patient's presentation of recurrent oral ulcers, acne, and genital ulcers is most consistent with Behçet disease. While her genital ulcers have been attributed to Herpes simplex type 2, there is no reported viral documentation. Behçet disease is a vasculitis of either small (most common), medium, or large vessels that involves multiple organs (summarized in the above table). Diagnosis is based on clinical findings (recurrent oral aphthous ulcers > 3 times/year) plus two of the following: eye lesions, recurrent genital lesions, skin lesions, or positive pathergy (> 2 mm papule forming 24-48 hours after inserting a 20-25 gauge needle 5 mm into skin). Treatment usually involves steroids and directed therapy towards the involved organ system. (Choice A) Aphthous stomatitis lesions are nearly identical to those of Behçet, but they are usually not associated with the acneiform lesions and genital ulcers seen in this patient. (Choice C) Coxsackie virus (hand-foot-and-mouth syndrome) occurs more commonly in children and usually presents with fever, sore throat, and oral or palmar/plantar lesions sparing the lips and gingiva. This patient's recurrent lesions and lack of palmar/plantar lesions makes this unlikely. (Choice D) Vulvar or scrotal scarring occurs very rarely in conditions other than Behçet disease. Also, given the patients family history of autoimmune disease and acne, Behçet disease is more likely than any other diagnosis. (Choice E) Nearly 50% of lupus patients can have lip and oral mucosal involvement with plaques (discoid lesions), erythematous lesions, punched out erosions, or ulcers. However, the oral ulcers (except for discoid lesions) are usually painless. This patient's painful ulcers make lupus unlikely. Educational objective: Patients with recurrent genital and oral ulcers combined with skin or ocular lesions likely have Behçet disease and require further evaluation. __________ References: Diagnosis/Classification Criteria for Behçet's Disease (http://www.ncbi.nlm.nih.gov/pubmed/21961081) New Insights in the Clinical Understanding of Behçet's Disease. (http://www.ncbi.nlm.nih.gov/pubmed/22187230)

A 25-Year-Old Woman Comes to the Physician Because of Multiple