Congenital deficiency of leukocyte-adherence glycoprotein D84.9

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

CDLG

Definition
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Congenital immune defect with disturbed leukocyte function and tendency to infection.

Clinical features
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Pyoderma gangraenosum, skin necrosis, ulcerations, impaired wound healing, gingivitis, stomatitis, otitis media, pneumonia, etc.

Therapy
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No causal therapy known. Symptomatic treatment of the skin changes and infections.

Progression/forecast
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30% lethality.

Literature
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  1. Bedlow AJ et al (1998) Pyoderma gangrenosum in a child with congenital partial deficiency of leucocyte adherence glycoproteins. Br J Dermatol 139: 1064-1067
  2. Kerkhof PCM, Weemaes CMR (1990) Skin manifestation in congenital deficiency of leukocyte-adherence glycoprotein (CDLG). Br J Dermatol 123: 395-401

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Last updated on: 29.10.2020