Leukocyte adhesion deficiency type 1 presenting with recurrent pyoderma gangrenosum and flaccid scarring

Pediatric Dermatology

Volumn 27, Issue 5, 2010, Pages 500-503

  Hinze, Claas H ^a   Lucky, Anne W ^a   Bove, Kevin E ^a   Marsh, Rebecca A ^a   Bleesing, Jack H ^a   Passo, Murray H ^a  

^a CINCINNATI CHILDREN'S HOSPITAL MEDICAL CENTER   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ADALIMUMAB; COLONY STIMULATING FACTOR; CYCLOPHOSPHAMIDE; CYCLOSPORIN; DAPSONE; HYDROCORTISONE; INFLIXIMAB; METHOTREXATE; MYCOPHENOLIC ACID 2 MORPHOLINOETHYL ESTER; PREDNISONE;

ARTICLE; CASE REPORT; CHILD; CLINICAL FEATURE; DRUG MEGADOSE; HUMAN; HUMAN TISSUE; LEUKOCYTE ADHESION DEFICIENCY; LEUKOCYTE ADHESION DEFICIENCY TYPE 1; MALE; PRIORITY JOURNAL; PYODERMA GANGRENOSUM; RECURRENT DISEASE; SCAR; SCHOOL CHILD; SKIN BIOPSY; SKIN DEFECT; SKIN ULCER; WOUND HEALING;

BIOPSY; CHILD; CICATRIX; FATAL OUTCOME; HUMANS; LEUKOCYTE-ADHESION DEFICIENCY SYNDROME; MALE; PYODERMA GANGRENOSUM; RECURRENCE; SKIN;

EID: 77957308818     PISSN: 07368046     EISSN: 15251470     Source Type: Journal    
DOI: 10.1111/j.1525-1470.2010.01260.x     Document Type: Article

References (17)

1. Callen J P. Pyoderma gangrenosum. Lancet 1998 351 : 581 585.
2. Stiehm ER, Ochs HD, Winkelstein JA. Immunologic disorders in infants & children, 5th ed. Philadelphia, PA : Elsevier Saunders, 2004 : xxi, 1512 p. 631 364.
3. Lekstrom- Himes JA, Gallin JI. Immunodeficiency diseases caused by defects in phagocytes. N Engl J Med 2000 343 : 1703 1714.
4. Arnaout MA. Leukocyte adhesion molecules deficiency: its structural basis, pathophysiology and implications for modulating the inflammatory response. Immunol Rev 1990 114 : 145 180.
5. Anderson DC, Schmalsteig FC, Finegold MJ et al. The severe and moderate phenotypes of heritable Mac-1, LFA-1 deficiency: their quantitative definition and relation to leukocyte dysfunction and clinical features. J Infect Dis 1985 152 : 668 689.
6. Malech HL, Hickstein DD. Genetics, biology and clinical management of myeloid cell primary immune deficiencies: chronic granulomatous disease and leukocyte adhesion deficiency. Curr Opin Hematol 2007 14 : 29 36.
7. Bedlow AJ, Davies EG, Moss AL et al. Pyoderma gangrenosum in a child with congenital partial deficiency of leucocyte adherence glycoproteins. Br J Dermatol 1998 139 : 1064 1067.
8. Davies KA, Toothill VJ, Savill J et al. A 19-year-old man with leucocyte adhesion deficiency. In vitro and in vivo studies of leucocyte function. Clin Exp Immunol 1991 84 : 223 231.
9. van de Kerkhof PC, Weemaes CM. Skin manifestations in congenital deficiency of leucocyte-adherence glycoproteins (CDLG). Br J Dermatol 1990 123 : 395 401.
10. Yong K, Addison IE, Johnson B et al. Role of leucocyte integrins in phagocyte responses to granulocyte-macrophage colony stimulating factor (GM-CSF): in vitro and in vivo studies on leucocyte adhesion deficiency neutrophils. Br J Haematol 1991 77 : 150 157.
11. Mates M, Kirkpatrick CH. A man with multiple infections with unusual organisms. Ann Allergy Asthma Immunol 1999 82 : 17 22.
12. Mansouri D, Adimi P, Mirsaedi M et al. Primary immune deficiencies presenting in adults: seven years of experience from Iran. J Clin Immunol 2005 25 : 385 391.
13. Weenig RH, Davis M D, Dahl PR et al. Skin ulcers misdiagnosed as pyoderma gangrenosum. N Engl J Med 2002 347 : 1412 1418.
14. Ley K, Laudanna C, Cybulsky MI et al. Getting to the site of inflammation: the leukocyte adhesion cascade updated. Nat Rev Immunol 2007 7 : 678 689.
15. Kim M, Carman CV, Springer TA. Bidirectional transmembrane signaling by cytoplasmic domain separation in integrins. Science 2003 301 : 1720 1725.
16. Martin P. Wound healing - aiming for perfect skin regeneration. Science 1997 276 : 75 81.
17. Peters T, Sindrilaru A, Hinz B et al. Wound-healing defect of CD18(-/-) mice due to a decrease in TGF-beta1 and myofibroblast differentiation. EMBO J 2005 24 : 3400 3410.