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.
Department of Dermatology, University Hospital of Nijmegen, The Netherlands.
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van de Kerkhof
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Weemaes
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