1. A case of 33-year-old house wife with Senear-Usher syndrome was reported.
2. Antiepithelial antibody in the sera of the patient was demonstrated by indirect IF staining technique. Bound IgG was present in the intercellular space of the prickle cell layer of the skin.
3. IgG was significantly high in the cantharidin blister fluid induced from uninvolved skin of the patient while IgA and IgM remained slightly higher levels.
4. Several immunological tests for lupus erythematosus gave negative results.
5. These data indicate that the Senear-Usher syndrome should be classified as a form of pemphigus. However, more cases should be further accumulated and immunologically investigated as an aid to clarify the relationship between lupus erythematosus and this syndrome and to elucidate the pathogenesis of the syndrome.