Leukocyte Adhesion Defects Types I & II

Key Features

• Recurrent serious infections

• "Cold" abscesses without pus formation

• Poor wound healing

• Gingival or periodontal disease (or both)

Clinical Findings

• Patients present with variably severe phenotypes, including

  • Recurrent serious infections

  • Lack of pus formation

  • Poor wound healing

  • Gingival and periodontal disease

• The hallmark is little inflammation and absent neutrophils on histopathologic evaluation of infected sites (ie, "cold" abscesses), especially when concurrent with neutrophilia, and expression of poor adherence to vessel walls

• The most severe phenotype manifests with infections in the neonatal period, including delayed separation of the umbilical cord with associated omphalitis

Diagnosis

• Striking neutrophilia demonstrated on laboratory evaluation

• Diagnosis is confirmed by flow cytometry analysis for CD18 (type I) or CD15s (type II)

Treatment

• Aggressive antibiotic therapy

• Fucose supplementation in type II has been reported with some success