Leukocyte adhesion deficiency: recurrent childhood skin infections.

Abstract

Leukocyte adhesion deficiency is a rare autosomal recessive disorder caused by the absent or severely reduced cell surface expression of a subfamily of leukocyte integrin molecular (beta 2 leukocyte integrins, CD11a/CD18, CD11b/CD18, CD11c/CD18). These molecules are required for the normal processes of leukocyte adherence and chemotaxis. Patients often die during the first years of life of a severe bacterial or fungal infection. We describe a 13-year-old boy with leukocyte adhesion deficiency who had intermittent cutaneous infections that were recalcitrant to treatment. Except for periodontitis, he had no other clinical problems. The diagnosis of leukocyte adhesion deficiency should be considered in patients with recurrent cutaneous infections that do not respond well to therapy, even in the absence of more serious manifestations. Evaluation should include flow cytometry studies to detect surface expression of the beta 2 leukocyte integrins on both resting and activated polymorphonuclear leukocytes.