title: Osteomyelitis in leukocyte adhesion deficiency type 1 syndrome.
abstract: Leukocyte adhesion deficiency type 1 (LAD-1) is a rare, inherited immunodeficiency that affects one per million people yearly and usually presents with recurrent, indolent bacterial infections of the skin, mouth, and respiratory tract and impaired pus formation and wound healing. A 13-year-old girl diagnosed LAD-I at the age of 7 years was brought to the Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, because of a draining plaque on the left leg for 2.5 years. She had recurrent skin infections and had been treated with repeated courses of different antibiotic combinations, with temporary responses, since 5 years of age. Examination revealed a 7 x 8 cm minimally erythematous hyperpigmented plaque with multiple draining sinuses on the left leg. Tissue culture yielded Pseudomonas aeruginosa. Flow cytometry showed CD18 (18.79%), CD11a (51.59%), CD11b (18.61%) and CD11c(10.60%). A plain radiography of the left leg revealed osteomyelitis. It is highly suggested that patients diagnosed mild to moderate LAD-1 with recurrent skin infection and simultaneous weak response to conventional therapy undergo (BMT) marrow transplant to prohibit subsequent life-threatening complications.
authors.affiliation: Bu Ali Immunology Research Center, Mashhad University of Medical Science, Mashhad, Iran. JabbariF@mums.ac.ir
authors.forename: Farahzad
authors.initials: F
authors.lastname: Jabbari Azad
authors.forename: Maryam
authors.initials: M
authors.lastname: Ardalan
authors.forename: Ali
authors.initials: A
authors.lastname: Hoseinpoor Rafati
authors.forename: Soheila
authors.initials: S
authors.lastname: Sotoudeh
authors.forename: Zahra
authors.initials: Z
authors.lastname: Pourpak
mesh_terms: D000293:Adolescent; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D016026:Bone Marrow Transplantation; D018821:CD18 Antigens / Q000096:biosynthesis; D005260:Female; D006801:Humans; D007866:Leg / Q000000981:diagnostic imaging / Q000382:microbiology; D018370:Leukocyte-Adhesion Deficiency Syndrome / Q000150:complications* / Q000378:metabolism / Q000601:surgery; D007962:Leukocytes / Q000378:metabolism; D010019:Osteomyelitis / Q000000981:diagnostic imaging / Q000209:etiology* / Q000378:metabolism; D011552:Pseudomonas Infections / Q000188:drug therapy / Q000209:etiology / Q000378:metabolism; D011550:Pseudomonas aeruginosa / Q000187:drug effects; D011859:Radiography; D017211:Treatment Failure
chemical_list: D000900:Anti-Bacterial Agents; D018821:CD18 Antigens