Diagnostic Information

Medical History

Before the onset of his oral lesions, the patient was in excellent health.  He had a 25 year history of gout for which he used diclofenac as needed. He also used celecoxib for joint pain in his knees.

Oral Findings

The patient had multiple irregular ulcerations of the posterior hard and soft palate, buccal mucosa, floor of mouth, and gingiva. (Figures 1-4) The superficial layer of the mucosa could be peeled away when firm lateral pressure was applied (positive Nikolsky sign).

Figure 1. Widespread ulcerations of the soft palate.

Figure 2. Vesiculoerosive lesions of the right buccal mucosa.

Figure 3. Diffuse ulcerations along the marginal gingiva.

Figure 4. Erosions of the floor of the mouth.

Other Clinical Findings

The patient also complained of tender, crusted ulcerations involving the left ear canal and nares. He denied the presence of any ocular or genital lesions. However, he did report the recent development of red splotches on his chest and an ulceration on his back. (Figure5)

Figure 5. Ruptured vesicle on the patient’s back.

Incisional Biopsy and Photomicrographs

An incisional biopsy was obtained from the edge of one of the ulcers, including some normal-appearing adjacent tissue. The mucosa was partially covered by stratified squamous epithelium, which showed loss of cohesion of the spinous epithelial cells (acantholysis). (Figure 6) The superficial epithelial layers were missing throughout much of the specimen, leaving behind one to several layers of basilar epithelial cells resembling a “row of tombstones.” (Figure 7) Rounded acantholytic cells (“Tzanck cells”) could be found above the attached basilar cells.

Figure 6. Medium power photomicrograph showing suprabasilar blister formation with loss of the superficial layers of the epithelium. The cells of the spinous layer show loss of cohesion (acantholysis) and float freely within the blister area.

Figure 7. High power photomicrograph showing loss of the upper epithelium with a remaining layer of basilar epithelial cells that resembles a “row of tombstones.”

Immunofluorescence Studies

Additional biopsy material from an uninvolved site was transported in Michel’s fixative (Poly Scientific, Bay Shore, NY 11706), frozen, and prepared for direct immunofluorescence studies. This specimen was positive for both IgG and C3 in the intercellular spaces between the spinous epithelial cells. (Figure 8)

Figure 8. Direct immunofluorescence highlights the presence of IgG in the intercellular spaces between the spinous epithelial cells.