Disorder of cellular immunity in pemphigus vegetans

Abstract

In an untreated 26-year-old female patient suffering from Neumann's pemphigus vegetans for 8 months (with neither preceding thymoma nor myasthenia gravis), several in vivo and in vitro phenomena of immunodeficiency involving both T and B cell system were disclosed. A diagnosis of defective immune state was established on the basis of the low IgG-serum level (though pemphigus antibodies were present), reduced T cell count in blood, weak PHA stimulation of lymphocytes, lack of skin reactivity to recall antigens and to most other bacterial antigens tested. Impaired chemotaxis and a deficient intracellular killing of Candida albicans by PMNL were also found. The conclusion is drawn that a combined disorder of both the B and T cell system is involved in the etiology of pemphigus vegetans, even when thymoma is ruled out.