Skin manifestations in congenital deficiency of leucocyte-adherence glycoproteins (CDLG).
In congenital deficiency of leucocyte-adherence glycoproteins (CDLG) there is an immunodeficiency with impaired leucocyte function and cutaneous and extracutaneous infections occur. In more than 30% of cases the condition has a fatal course. We report the skin manifestations of three siblings with CDLG in which areas of skin necrosis occurred that resembled pyoderma gangrenosum.
van de Kerkhof
P C
PC
Department of Dermatology, University Hospital of Nijmegen, The Netherlands.
Weemaes
C M
CM
D000328:Adult; D005260:Female; D005734:Gangrene; D006801:Humans; D018370:Leukocyte-Adhesion Deficiency Syndrome; D008297:Male; D008661:Metabolism, Inborn Errors / Q000473:pathology*; D012867:Skin / Q000473:pathology; D012883:Skin Ulcer / Q000378:metabolism* / Q000473:pathology