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Diffuse proliferative glomerulonephritis
24%
15/63
Neonatal herpes
27%
17/63
Pott disease
3%
2/63
Sideroblastic anemia
5%
3/63
Thrombosis
38%
24/63
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This patient is presenting with recurrent, painful oral and genital aphthous ulcers and erythema nodosum (tender nodules on the legs), suggesting the diagnosis of Behcet syndrome. This syndrome may be complicated by thrombosis.Behcet syndrome occurs more commonly in young adults of Turkish, Middle Eastern, or Asian descent. The diagnosis is made clinically with characteristic findings including recurrent, painful oral and genital aphthous ulcers, uveitis, arthritis, and non-specific skin lesions such as erythema nodosum. The main causes of mortality are thrombosis and vasculitis. A biopsy is not necessary for diagnosis, but it may show a vasculitis of different-sized vessels. Treatment may include topical corticosteroids as well as colchicine.Yazici et al. review the evidence regarding the diagnosis and treatment of patients with Behcet syndrome. They discuss how the haplotype B51 is associated with this disease. They recommend understanding the pathophysiology in order to better treat this disease.Figure/Illustration A is a clinical photograph showing inflammation of the uvea, the iris surrounding the pupil, and the ciliary body (red circle). This finding of anterior uveitis is commonly seen in patients with Behcet syndrome.Incorrect Answers:Answer 1: Diffuse proliferative glomerulonephritis is the most common renal disease of systemic lupus erythematosus (SLE). Oral ulcers can be seen in SLE, but they are usually painless. Other symptoms of SLE include malar rash, photosensitivity, arthritis, muscle stiffness, and serositis. Treatment of lupus may include hydroxychloroquine.Answer 2: Neonatal herpes is a potential complication of active genital herpes in pregnancy. Herpes simplex can cause oral and genital ulcers and uveitis. These lesions will first appear as grouped vesicles. In this case, the Tzanck test was negative (no multinucleated giant cells under microscopy). Treatment is with acyclovir.Answer 3: Pott disease is a form of vertebral osteomyelitis caused by Mycobacterium tuberculosis. Risk factors for tuberculosis include malnourishment, living in crowded conditions, and recent immigration. Cutaneous tuberculosis is rare, and this patient lacks the classic pulmonary and systemic symptoms of tuberculosis (hemoptysis, night sweats, and weight loss). Treatment is with combination therapy usually involving rifampin and isoniazid among other agents.Answer 4: Sideroblastic anemia may be seen in vitamin B6 (pyridoxine) deficiency. Oral manifestations of vitamin B6 deficiency include stomatitis, glossitis, and cheilosis. Vitamin B6 deficiency is also associated with peripheral neuropathy and decreased niacin synthesis. Treatment is with vitamin supplementation.Bullet Summary: Thrombosis is a common complication of Behcet disease.
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