Leukocyte adhesion deficiency

Overview

Board takeaway: Refer early destructive periodontitis to immunology and periodontics; infections may lack pus despite high circulating neutrophil counts. An integrin (CD18) defect prevents neutrophils from leaving blood vessels.

How does Leukocyte adhesion deficiency affect dental management?

• Coordinate promptly: immunology, hematology, and periodontics.
• Plan infection treatment and procedural prophylaxis: a high circulating neutrophil count does not indicate effective tissue defense; plan with the medical team.
• Periodontal care: individualized caregiver-supported plaque control and maintenance; chlorhexidine may be an adjunct when delivery is safe.
• Treatment limit: severe disease may remain refractory to debridement and antibiotics because the immune defect persists.
• Rehabilitation: Extraction of hopeless teeth; prosthetic replacement

Exam pearl

What is Leukocyte adhesion deficiency?

A group of inherited immunodeficiency disorders in which defective leukocyte adhesion or migration prevents white blood cells from reaching infected tissues effectively, leading to recurrent infections and poor wound healing.

What are the main systemic features of Leukocyte adhesion deficiency?

• Genetics: Autosomal recessive; LAD-I (ITGB2/CD18) is the most common type
• Systemic clues: Persistent leukocytosis (often over 25,000); poor wound healing; omphalitis
• LAD-II: Bombay blood type with intellectual disability
• LAD-III: bleeding from platelet dysfunction
• Prognosis: Severe forms are fatal in early childhood without hematopoietic stem cell transplant

What oral findings occur in Leukocyte adhesion deficiency?

• Severe gingival inflammation and rapid bone loss around primary teeth soon after eruption
• Premature loss of primary and permanent teeth
• Oral ulcers with slow healing

For Leukocyte adhesion deficiency, why are neutrophils high but infections lack pus?

• CD18 defect prevents neutrophil adhesion and exit from vessels
• Blood neutrophilia despite tissue neutrophil deficiency and absent pus

An infant has delayed umbilical cord separation, skin infections without pus, and severe gingival inflammation with bone loss around newly erupted primary teeth. What is the likely diagnosis?

Leukocyte adhesion deficiency type I, an autosomal recessive ITGB2 (CD18) defect.

Neutrophils cannot adhere to and exit blood vessels. This explains the combination of a very high blood neutrophil count, tissue neutrophil deficiency, and absent pus.

What other conditions can cause prepubertal periodontitis?

• Papillon–Lefèvre syndrome
• Cyclic and congenital neutropenia
• Chédiak–Higashi
• Hypophosphatasia
• Down syndrome
• LCH and leukemia

Sources

• AAPD dental guidance: AAPD: Systemic diseases and syndromes that affect the periodontium
• Supplemental systemic features: MedlinePlus Genetics: Leukocyte adhesion deficiency type 1
• Supplemental study guide: Pediatric Dental Study Guide: Leukocyte Adhesion Deficiency (LAD)
• Periodontal Conditions in Pediatric Dental Patients
• Systemic Diseases and Syndromes that Affect the Periodontium