Vulvar Lesions
Article Outline
[Ann Emerg Med. 2011;57:329.]
An 11-year-old healthy girl presented to the emergency department with 4 days of fever, knee arthralgias, and painful genital lesions. Her physical examination was notable for multiple purplish vulvar ulcerations; there was no perianal, oral, or conjunctival involvement (Figure).
Figure.
Vulvar lesions.
Used with permission of Monika Goyal, MD, Children's Hospital of Philadelphia, Department of Pediatrics, Division of Emergency Medicine, Philadelphia, PA.
Diagnosis
Behçet's disease. Diagnostic evaluation of this patient included bacterial and herpes simplex virus cultures from the lesions, whose results were negative. Serologic testing for Epstein-Barr virus, cytomegalovirus, herpes simplex virus, and Mycoplasma pneumoniae was also negative, as was HLA-B51 and an inflammatory bowel disease panel (anti-neutrophil cytoplasmic antibody and anti–S accharomyces cerevisiae antibody). A C-reactive protein level was 8.4 mg/dL, and a sedimentation rate was 23 mm/hour. Ophthalmologic examination did not reveal evidence of uveitis. A presumptive diagnosis of Behçet's disease was made, and treatment with colchicine and prednisone was instituted. The vulvar ulcerations promptly resolved with therapy and did not recur.
The differential diagnosis of painful genital ulcers in children includes herpes simplex virus, chancroid, and bacterial or fungal infections, as well as noninfectious causes, which include systemic lupus erythematosus, Crohn's disease, neoplasm, and Behçet's disease.1
Behçet's disease is a multisystem vasculitis classically associated with mucocutaneous lesions and variable clinical manifestations, including recurrent oral aphthae, genital ulcers, skin lesions, uveitis, thrombophlebitis, and gastrointestinal and central nervous system involvement. Patients with Behçet's disease often enter spontaneous remission, although the disease is more severe in younger patients.2 Treatment consists of corticosteroids and immunomodulators, such as colchicine or azathioprine.3
References
- Pediatric vulvovaginal disorders: a diagnostic approach and review of the literature. Gynecology. 2009;31:850–862
- . Behçet's syndrome. Best Pract Res Clin Rheumatol. 2008;22:793–809
- Management of Behçet disease: a systemic literature review for the European League Against Rheumatism evidence-based recommendations for the management of Behçet disease. Ann Rheum Dis. 2009;68:1528–1534
For the diagnosis and teaching points, see page 361.
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PII: S0196-0644(10)00479-8
doi:10.1016/j.annemergmed.2010.05.011
© 2010 Published by Elsevier Inc.
