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February 1985

Darier-White Disease: A Multifaceted Immunodeficient State?

Author Affiliations

Department of Immunology University Hospital Medical School 29279 Brest Cédex, France

Arch Dermatol. 1985;121(2):170. doi:10.1001/archderm.1985.01660020028008

To the Editor.—  Darier-White disease has recently acquired a new interest on the basis of an immunological dysfunction, even though no consistent and specific abnormality has yet been demonstrated. The evidence for this has been comprehensively reviewed in the Archives.1 So, we would like to report the results of the study of such a patient, emphasizing three features.

Findings.—  We found that the patient's serum inhibited killing of Staphylococcus aureus by neutrophils from three normal controls (86.2% ± 2.3% of killing with control sera, as opposed to 40.3% ± 4.0% with the patient's serum). This disappeared after four months from the onset of the disease. Other classical neutrophil function tests, including phagocytosis, nitroblue tetrazolium reduction, migration, and adherence, were all normal. These results suggest a subtle neutrophil abnormality, are in accordance with those published so far,2,3 and justify the regular study of neutrophil functions in these patients.The