ABSTRACT
INTRODUCTION: Leukocyte adhesion deficiency syndrome (LAD) is an altered phagocytic disorder characterised by the deficiency of one or several integrins which are included within the adhesion molecules group and cell surface receptors superfamily. OBJECTIVE: To describe the clinical features of a rare primary immunodeficiency case. CLINICAL CASE: A nineteen days-old male newborn was referred to the pediatrics infectology service because a 15 days clinical course characterised by delayed cord detachment; fever and skin lesions in several arcas that evolved to, cellulitis and dermal necrosis: Then he was admitted with the diagnosis of septicemia secondary to omphalitis. There were a partial response to antimicrobial treatment. Thereafter he had recurrent respiratory and gastrointestinal fungal and bacterial infections. Then he suffered psychomotor impairment and severe malnutrition. The patient died because septicemia at 5 months-old. WBC counts showed persistent leukocytosis between 42,000 and 133,000 cells/mm3, mostly neutrophils (64%-88%). We also found defective neutrophil quimiotaxis. By flow cytometer it was detected CDII/18 adhesins deficiency. Otherwise immunological, bone marrow biopsy and viral tests were, normal. CONCLUSIONS: Although its prevalence is rare, leukocyte adhesion defects must be considered in those patients with delayed cord detachment, recurrent severe infections and both persistent and elevated neutrophilia and with other primary and secondary immunodeficiencies previously discarded.
