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PART fI
Soft Tissue Lesions
are pemphigus vulgaris, pemphigus vegetans, pemphigus foliaceus, and pemphigus erythematosus. They have similar immunologic findings, which are diagnostic (see Table 6-1).
Features Pemphigus affects men and women approximately equally, and the vast majority of patients are white. In one report, 17 74% of the patients with the vulgaris or vegetans type were Jewish, approximately 98% of the patients were over 31 years of age at the time of onset, and 80% of cases of the vulgaris variety (by far the most common type) first OCCUlTed intraorally. Laskaris and Stoufi J 8 reviewed the rare cases of pemphigus vulgaris in children. The lesions of pemphigus vulgaris may appear as areas of erosion, but more often they are seen as ulcers, bullae, or areas of sloughing mucosa or skin (Fig. 6-9 and Plate C, 5). Diffuse erythematous involvement of the gingiva has been reported but is not the typical manifestation of the disease. In the typical case, initial lesions occur orally, followed by skin lesions.
The histologic study of intact vesicles or bullae reveals an intraepithelial defect. The individual cells separate from one another, and a pooling of fluid occurs. The basal layer of epithelial cells remains in position on the basement membrane.
Differential Diagnosis All the vesiculobullous conditions listed at the beginning of this chapter should be considered in the differential diagnosis. As a general rule, the pemphigus bulla is smaller than the bulla in benign mucous membrane pemphigoid and considerably larger than those seen in the viral diseases such as herpes and hand-foot-and-mouth disease. Special diagnostic procedures Immunofluorescence studies of sera and biopsy specimens reveal antibodies confined to the intercellular substance of epithelium and intercellular deposits of IgO (Fig. 6-9). The sera from more than 95% of pemphigus patients with active disease contain intercellular epithelial antibodies. During early stages of the disease or during remission. the
A
c
B
Fig. 6-9. Pemphigus. A and B, Clinical views of the same case. The mucosa of the lower lip is much more severely involved than the buccal mucosa. C, Direct immunofluorescence test on oral biopsy specimen from patient with pemphigus revealing TgG deposits intercellularly in the epithelium. Epithelial intercellular antibodies are also seen in the serum. Both are diagnostic. (A and B courtesy M. Lehnert, Minneapolis.)