Answer, Image 6:
IMMUNOLOGIC/GENETIC (leading to INFECTIOUS complications)
The gross photograph demonstrates an open wound of the abdomen, following surgical treatment of appendicitis and subsequent surgery to treat complications of poor wound healing, including debridement of necrotic tissues. Evidence of ongoing wound infection can be seen at the top of the wound.
Leukocyte adhesion deficiency (LAD), Type I is caused by autosomal recessive mutations affecting the common b subunit (CD18) shared by three cell adhesion receptors with unique a subunits (LFA-1 or CD11a; complement receptor CR3 or CD11b; and p150,95 or CD11c). Patients with LAD have histories of delayed separation of the umbilical cord, recurrent skin infections and otitis media, pneumonia, and poor wound healing, all of which were exhibited by this patient. Lack of CD18 leads to poor neutrophil function. The severe phenotype in LAD patients emphasizes the importance of neutrophil function in wound healing processes. LAD has been cured by bone marrow transplantation.
Diagnosis:
Leukocyte adhesion deficiency, Type I