Lymphocyte function antigen-1 [LFA-1] defect

This code represents a primary immunodeficiency disorder characterized by a defect in the lymphocyte function-associated antigen-1 (LFA-1) protein. This defect impairs the ability of immune cells, particularly leukocytes, to adhere to endothelial cells and other immune cells, leading to recurrent, severe bacterial and fungal infections, particularly of the skin and mucous membranes, and impaired wound healing.

Use this code for patients diagnosed with Leukocyte Adhesion Deficiency Type I (LAD-I), which is caused by a genetic defect in the ITGB2 gene encoding the CD18 subunit of LFA-1. Documentation should clearly state the diagnosis of LFA-1 defect or LAD-I, often supported by genetic testing results or characteristic clinical findings such as omphalitis, recurrent infections without pus formation, and impaired wound healing.

• Ensure documentation specifies "LFA-1 defect" or "Leukocyte Adhesion Deficiency Type I" to differentiate from other adhesion molecule defects.
• Sequence this code as a primary diagnosis when it is the reason for the encounter or hospitalization.
• Consider co-reporting codes for associated manifestations such as recurrent infections (e.g., cellulitis, periodontitis) or failure to thrive, if applicable.