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.
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).
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)
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.
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)