A Case of Leukocyte Adhesion Deficiency (LAD) Presenting with Nasal Septum Perforation and Necrotizing Ulcer on Ala Nasi

Author:

Tezcan Ilhan1,Yel Leman1,Önerci Metin2,Sanal Özden1,Ersoy Fügen1,Berkel A. Izzet1

Affiliation:

1. Divisions of Immunology Department of Pediatrics, Hacettepe University Children's Hospital, Ankara, Turkey

2. Ear-Nose-Throat Department, Hacettepe University Medical School, Ankara, Turkey

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.

Publisher

SAGE Publications

Subject

Otorhinolaryngology

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