You ponder this information and note that your patient has no oral lesions currently but apparently has a history of some oral lesions. You review your records indicating a history of recurrent perioral rash but do not find a history of oral lesions. However, the consultant remains constant in his diagnosis.
You review the results of the ED laboratory evaluation:
- Gonorrhea/chlamydia DNA probe negative
- Herpes culture and herpes immunoglobulin M (IgM) negative
- Rapid plasma reagin (RPR) nonreactive
- Serum pregnancy negative
- Urine drug screen negative for PCP, benzodiazepines, amphetamines, THC, opiates, barbiturates, methadone, tricyclic antidepressants
- Complete blood count normal; white blood count 9600 with a normal differential
As the family physician, you interpret these results and support the clinical plan for this patient, pending further changes in the clinical picture.
TABLE 1
Differential diagnosis of vulvar ulcers in sexually inexperienced females
| Sexual abuse |
| Herpes simplex virus infection |
| Behçet’s disease |
| Epstein-Barr viral infection |
| Crohn’s disease |
| Pilonidal disease |
| Hidradenitis suppurativa |
Next contact—ED visit 2 days later
The patient’s vaginal pain is worsening and she cannot void. Large ulcers with adhered pus are on the left labia; a smaller lesion is on the right labia. There are also questionable lacerations of the posterior fourchette. She is catheterized and 300 mL urine is emptied from the bladder. A gynecology consultation is obtained by the ED physician with your concurrence.
Q: This patient has had an extensive work up. Is there any other diagnostic testing you would consider?
A: ____________________________________________________________ ______________________________________________________________ ______________________________________________________________
Q: Is this Behçet’s disease?
A: ____________________________________________________________ ______________________________________________________________ ______________________________________________________________
Details of Behçet’s disease
Behçet’s disease is most common in the third or fourth decade of life. It has an association with the human leukocyte antigen HLA-B51 and HLA-B5 allele. Vascular injuries, hyperfunction of neutrophils, and autoimmune responses lead to the clinical findings. Behçet’s disease is presumed to be an autoimmune disease, with the primary lesion being related to the vasculitis.
Clinical criteria of Behçet’s
Diagnosis of Behçet’s disease is based on clinical criteria. No single test can determine if a patient has Behçet’s.
An International Study Group of physicians was convened to develop a set of guidelines to diagnose Behçet’s disease. These criteria include recurrent, painful oral ulceration with at least 2 other symptoms (recurrent genital ulcers, eye lesions, skin lesions, and positive pathergy test) (TABLE 2). These diagnostic criteria may be accessed at the website of the American Behçet’s Disease Association, www.behcets.com.
Prevalence of signs
Oral ulcerations are the presenting signs in about 70% of cases. These ulcers may remain for as long as 3 weeks. In women, the genital lesions are often present in the vulvovaginal region; in men, they often appear in the scrotal area. Ocular complaints occur in about one-half of patients, sometimes with photophobia, watering, and blurred vision. The most common skin lesions are pseudofolliculitis and erythema nodosum.
The pathergy test uses a sterile needle to make a skin prick. The test result is positive if an aseptic erythematous nodule or pustule (>2 mm in diameter) occurs within 24 to 48 hours, indicating neutrophil hyperfunction.
Differential diagnosis
Skin, joint, gastrointestinal, vascular, and central nervous systems may also be involved. The differential diagnosis includes chronic oral aphthosis, herpes simplex virus infection, Sweet’s syndrome, ankylosing spondylitis, inflammatory bowel disease, and multiple sclerosis (TABLE 3).
Laboratory findings and treatment
Examination of the blood can reveal nonspecific findings consistent with inflammation, including elevated C-reactive protein, erythrocyte sedimentation rate, and positive markers for autoimmune diseases.
Treatmentdepends upon the particular symptoms and clinical findings and must be coordinated with the various specialists involved.
Topical steroids are used for oral and genital ulcers as well as ocular lesions.
Oral steroids are useful for gastrointestinal and neurological symptoms but may also help with skin, joint, vascular, and ocular symptoms.
Colchicine may help alleviate oral and genital ulcers as well as skin, ocular, and joint problems.
Topical tetracycline may be used to treat oral ulcers.
Anticoagulants may be indicated for vascular disturbances and progressive central nervous system lesions.
Cytotoxic and antirheumatic agents may treat vascular, ocular, neurological, and joint manifestations.
Q: If the patient does not meet the criteria for Behçet’s disease, what are the next steps?
A: ____________________________________________________________ ______________________________________________________________ ______________________________________________________________ ______________________________________________________________ ______________________________________________________________
TABLE 2
Diagnostic criteria of Behçet’s disease
| Recurrent oral ulceration |
|
| AND AT LEAST 2 OTHER SYMPTOMS: |
| Recurrent genital ulcers |
| Eye lesions |
|
| Skin lesions |
|
| Positive pathergy test |
|
| Adapted from Sakane et al, N Engl J Med 1999.1 |