Pemphigus herpetiformis is a separate form of pemphigus with its own features. Clinically it resembles dermatitis herpetiformis Duhring. Periodically there is an extraordinary violent itching. Histologically pemphigus herpetiformis shows an eosinophilic spongiosis. The important acantholysis can only be found by repeated biopsies. Because of the acantholysis and the intercellular autoantibodies, demonstrated by direct and indirect immunfluorescence, this dermatosis belongs to the group of pemphigus. The therapeutical response to 4,4'-diaminodimethyl-diphenylsulfone (DDS)--a similarity to dermatitis herpetiformis Duhring--is especially characteristic. Our own observation gives occasion to characterize this pemphigus variant, which is repeatedly described in the literature under different designations, as pemphigus herpetiformis.