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Pyoderma gangrenosum in a child with congenital partial deficiency of leucocyte adherence glycoproteins
Author(s) -
A. J. Bedlow,
E. Graham Davies,
A.L.H. Moss,
Naomi Rebuck,
Adam Finn,
R.A. Marsden
Publication year - 1998
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1046/j.1365-2133.1998.02567.x
Subject(s) - pyoderma gangrenosum , neutrophilia , medicine , cd11a , leukocyte adhesion deficiency , cd18 , leukocytosis , immunology , inflammation , prednisolone , colchicine , pyoderma , integrin , pathology , dermatology , flow cytometry , integrin alpha m , disease , receptor
Congenital deficiency of β2 integrin leucocyte adhesion molecules is a rare immunodeficiency and is often fatal. Neutrophils are unable to bind to ligands on the endothelium, and so cannot leave the circulation during inflammation or infection. When leucocyte adhesion deficiency (LAD) is caused by abnormally low expression of β2 integrins, it is termed LAD type 1. We describe a 5‐year‐old girl with a history of recurrent bacterial infections since early childhood who developed necrotic skin ulcers resembling pyoderma gangrenosum and a persistent circulating neutrophilia. Histologically, the lesions showed deep ulceration with a diffuse lymphohistiocytic infiltrate, but with a relative sparsity of neutrophils. Subsequent investigation revealed a complete absence of CD11a/CD18 β2 integrins on the surface of the patient's neutrophils, confirming the diagnosis of LAD type 1. The ulcers responded to treatment with oral prednisolone and colchicine.