Brain Abscess in a Child with Leukocyte Adhesion Defect: An Unusual Association

Chronic mastoiditis associated with leukocyte adhesion deficiency type 1: A case report

“…The incidence of this disease is not well known. [3,9] The disease mainly affects the skin and mucosa, and may result in fatal bacterial infections. Due to the lack of inflammation, necrotic lesions develop, and wound healing is delayed.…”

“…First findings are delayed separation of the umbilical cord and omphalitis. [3,9] There is no increased proclivity to viral infections, but an increase in infections with Staphylococcus aureus, gram (-) enteric microorganism and fungi can be seen. Recurrent otitis media, perirectal abscess, cellulitis, chronic periodontitis are other commonly seen infections.…”

“…Recurrent otitis media, perirectal abscess, cellulitis, chronic periodontitis are other commonly seen infections. [3,9] Pseudomonas Aureginosa and Escherichia Coli, which are gram (-) microorganisms, were cultured from the infected tissue excised during surgery. On histopathologic examination, neutrophils are not observed in the tissue samples that are taken from the infected field.…”

“…[8] Leukocyte adhesion deficiency type 1, which is inherited in an autosomal recessive manner, is characterized by delayed separation of the umbilical cord, omphalitis, recurrent periodonditis and gingivitis, pyogenic bacterial infections (chronic otitis media, pneumonia, peritonitis, cellulitis), necrosis and ulcerations of the skin. [3] With informed consent obtained from her parents, we report the case of a six-year-old girl with LAD type 1 who presented with chronic mastoiditis and the treatment of this unusual presentation.…”

“…[1,2] Margination, rolling, adhesion and migration are the main steps necessary for leukocytic infiltration to inflammatory tissue, and are dependent on fully functional adhesion molecules. [3] Deficiencies of these molecules result in inadequate neutrophil migration and inflammatory response, termed leukocyte adhesion deficiency (LAD), and these diseases are classified as primary immunodeficiency syndromes.…”