Darier's disease: an immunologic study.

ABSTRACT

Darier's disease is frequently complicated by bacterial skin infections and occasionally by Kaposi's varicelliform eruption. Postulating that defective host immunologic competence might explain these infections, humoral and cell-mediated immunity (CMI) were evaluated in four patients. Humoral immunity was normal as demonstrated by quantitative immunoglobulins, isohemagglutinins, direct skin immunofluorescence, and B-cell counts. The CMI was evaluated by standard delayed type hypersensitivity skin tests, T-cell counts, lymphocyte transformation assays, macrophage inhibition factor (MIF) assays, and skin windows. Blunted lymphocyte blastogenesis, MIF, and skin window response indicated depressed CMI. Polymorphonuclear leukocyte chemotaxis and phagocytosis were normal.