Abstract
We report on a 3 month-old female patient having leukocyte adhesion deficiency (LAD) of severe phenotype presenting with perforation of the nasal septum and ulceration on the right ala nasi. Laboratory investigations revealed marked leukocytosis, defective neutrophil adherence to glass beads and chemotaxis, decreased NK cell cytotoxicity, and undetectable expression of CD18 on lymphocytes. We conclude that the possibility of this entity should be kept in mind in the presence of such a necrotic lesion in early childhood.
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