A review of 124 cases of bullous pemphigoid was made and the clinical findings correlated with the results of indirect immunofluorescence. A circulating IgG basement membrane zone (BMZ) antibody was detected in the serum of eighty-nine (72%) of the patients. Antibody titres in these patients did not correlate with the extent of the disease or with the presence of mucous membrane lesions. Indeed, oral lesions were more common in the thirty-five sero-negative patients with 17% of such patients being affected compared to only 10% of those with positive indirect immunofluorescence findings. This difference, however, was not statistically significant. Concurrent malignant disease (i.e. malignancy occurring within 6 months of pemphigoid being diagnosed) occurred in eight of the seronegative group (23%) and in only four of the eighty-nine seropositive cases (4%); this difference was significant. The association of bullous pemphigoid and malignant disease still remains controversial, but those who believe that the relationship is coincidental must now explain why concurrent malignant disease is more common in patients who have bullous pemphigoid and negative indirect immunofluorescence findings.
Summary
Sera from 50 untreated patients with bullous pemphigoid were examined by both the customary method of indirect immunofluorescence using anti IgG conjugate and by indirect complement immunofluorescence using anti C3 conjugate to detect circulating antibasement membrane zone antibodies. A circulating antibasement membrane zone antibody could be detected by the IgG method in 58% and by the C3 method in 76%. Sera from six patients with cicatricial pemphigoid examined in the same way showed a circulating antibasement membrane zone antibody in one by the IgG method but in three by the C3 method of indirect immunofluorescence. Sera from ten patients with active herpes gestationis contained anti‐basement membrane zone antibody demonstrable by the C3 method in every case and by the IgG method of indirect immunofluorescence in one of these. Basement membrane zone bound IgG, or more commonly C3 in a linear pattern, was shown by direct immunofluorescence in all patients with bullous or cicatricial pemphigoid from whom adequate biopsy material was obtained.
The immunopathological similarities of bullous pemphigoid, cicatricial pemphigoid and herpes gestationis are stressed, and the usefulness of indirect complement immunofluorescence in their diagnosis is emphasized.
Summary
The clinical, histological and immunopathological details of three cases with a d‐penicillamine‐induced eruption are presented. The skin changes included (1) pemphigus erythematosus, (2) pemphigus foliaceus, (3) a bizarre localized pruritic papular eruption, the immunopathology of which was characteristic of lupus erythematosus and (4) fragility due to loss of dermal collagen. Circulating IgA was abnormally low in two cases and circulating immune complexes were demonstrated in the third case.
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