Diagnosis: Behçet's Disease
PORTLAND, ORE. — The patient's oral lesions began with minor induration and ulceration along the closure line of teeth on the buccal mucosa, according to Dr. Bruce D. Ragsdale, who presented the case at the annual meeting of the Pacific Dermatologic Association.
Fever and chills set in with gingival, oral mucosal, and tongue ulcerations. The patient had multiple erythematous, dome-shaped, pustular lesions on proximal extremities plus a few on the trunk, noted Dr. Ragsdale. “Oral lesions start as painful fever blisters that come to a head and burst.”
The patient lost 15 pounds because tongue pain affected his ability to eat. Family history revealed that his grandfather and cousin had Behçet's disease. His white blood cell count was 23.9 pg/mL, with a morphonuclear neutrophil count of 89, a lymphocyte count of 5, and a mononucleocyte count of 7%, with no eosinophils or basophils. Tests for antinuclear antibody, mononucleosis, and HIV were negative. The initial differential diagnosis was herpes simplex or Behçet's disease. The man was hospitalized and started on 20 mg of prednisone and colchicine 0.6 mg t.i.d.
Dr. Ragsdale, founder and director of a dermatopathology practice in San Luis Obispo, Calif., selected a shoulder lesion, not yet ulcerated, for biopsy. Histopathology revealed that pus filled the partly destroyed chamber of a follicle, and that a few bacteria—but no fungal agents—occupied keratin debris in the follicular abscess. Nearby was a diffuse infiltrate of neutrophils in papillary dermis that resembled the neutrophil array of Sweet's syndrome, associated with some hypereosinophilic collagen. No vasculitis was evident. Based on the biopsy and the clinical context, Dr. Ragsdale determined that the man had familial Behçet's disease.
“Aphthous stomatitis is a major criterion and frequently the first symptom [in 64%-71% of patients], and is almost always present during the course of the disease,” Dr. Ragsdale noted. “It may precede other manifestations by many years. Genital and perianal aphthae tend to be larger [and] deeper, with regular margins, and [are] more painful than oral lesions.”
The International Study Group criteria for the diagnosis of Behçet's disease requires aphthous (idiopathic) oral ulceration that recurs at least three times in a 12-month period, plus any two of the following: recurrent genital ulceration, eye lesions, cutaneous lesions, or a positive pathergy test.
The patient's prednisone dosage was increased to 40 mg daily prior to his hospital discharge. He continues taking prednisone and colchicine, and finds that his oral lesions return when he takes less than 20 mg of prednisone.
Histopathology image (right) shows pus filling the partly destroyed chamber of a follicle.
Source Photos Courtesy Dr. Bruce D. Ragsdale
This shoulder lesion, shown before it ulcerated, yielded the biopsied tissue.