Treatment

The plan for treatment included performin and indirect IMF staining to confirm the diagnosis of pemphigus vulgaris, as well as increasing the prednisone dosage to 60 mg p.o. daily, taken as pills. He was also started on ranitidine 150 mg p.o. daily for GI prophylaxis and given calcium and vitamin D supplements. In addition, he started on CellCept, (mycophenolate mofetii) 500 mg p.o. b.i.d., for 1 week. This was then increased to 1000 mg p.o. in the morning and 500 mg at night for 1 week, and was again increased to 1000 mg b.i.d. Also included in the treatment plan was a referral to Ophthalmology and regular blood and liver lab tests.

Excisional Biopsy

The two biopsies were read by Dr. Oda and Dr. Morton of the Oral Pathology Biopsy Service at the University of Washington. The first biopsy was submitted in formalin for H & E staining and the second biopsy was submitted in transport medium for direct immunofluorescence staining. The latter biopsy was read by Dr. Morton and was the more specific of the two specimens. The direct immunofluorescence antibody staining was positive for IgG (Figure 7) and C3 (Figure 8) antibodies and was also focally positive with antibodies to fibrinogen. The positive IgG and C3 staining was pericellular and intracellular, involving the epithelial cells above the basal cell layers. The H & E stained frozen section (Figures 5 & 6) showed evidence of a split above the basal cell layer as well as areas of acantholysis. The connective tissue was infiltrated by lymphocytes, plasma cells, and neutrophils.

Figure 5 Low power (x100) Histology view of the H & E stained frozen section demonstrating a small punch biopsy with supra basilar epithelial split and inflamed underlying connective tissue.

Figure 6 Low power (x200) Histology view of the H & E stained frozen section demonstrating a small punch biopsy with supra basilar epithelial split and mild acantholysis of the middle spinous layer.

Figure 7 Low power (x200) view of a direct Immunoflourescent stain demonstrating positive staining with C3 antibody with a chicken wire intercellular deposits in the deep spinous layer.

Figure 8 Low power (x200) view of a direct Immunoflourescent stain demonstrating mildly positive staining with IgG antibody with a chicken wire intercellular deposits between the spinous layer cells.