IMMUNOPATHOLOGICAL INVESTIGATIONS IN THE SENEARUSHER SYNDROME (COEXISTENCE OF PEMPHIGUS AND LUPUS ERYTHEMATOSUS)

Abstract

SUMMARY.— In 5 of 6 cases of the Senear-Usher syndrome, in skin specimens from erythematous facial lesions, the immunofluorescence method demonstrated immunoglobulins and in vivo-fixed complement in the dermoepidermal junction, which is a finding characteristic of lupus erythematosus. Antibodies against nuclei were also present in all but one case, and in one there was coexistent systemic lupus erythematosus with positive LE phenomenon. In all 6 cases of the SU syndrome the presence of IgG as well as C′3a, C′4 was demonstrated in the intercellular spaces of the epidermis; this is a finding characteristic of pemphigus. In 6 cases of pemphigus vulgaris, which served as controls, neither immuno-globulins nor in vivo-fixed complement could be demonstrated in the dermoepidermal junction. These investigations suggest that the SU syndrome could, at least in the vast majority of cases, result from the coexistence of pemphigus and lupus erythematosus.