Darier's disease: an immunologic study

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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 4 patients. Humoral immunity was normal as demonstrated by quantitative immunoglobulins, isohemagglutinins, direct skin immunofluorescence and B[bone marrow-derived]-cell counts. The CMI was evaluated by standard delayed type hypersensitivity skin tests, T[thymus-derived]-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.

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