Defective expression of HLA class I and CD1a molecules in boy with Marfan-like phenotype and deep skin ulcers - 11/09/11
Abstract |
We report the case of a boy with low expression of HLA class I molecules on peripheral blood mononuclear cells, which is associated with immunodeficiency. The patient, who had a Marfan-like phenotype, had chronic deep skin ulcers and sinobronchiectasis. Immunohistologic examination of the ulcerated skin showed a dense perivascular infiltrate composed of normal mature lymphocytes and macrophages. All cells in the infiltrate showed an apparently normal expression of HLA class I molecules, but intraepidermal dendritic Langerhans' cells were negative for CD1a, an antigen that is a highly specific marker for these cells and is abundantly expressed in some self-healing forms of cutaneous lesions. It is therefore speculated that a defective expression of CD1a molecules can contribute to the chronic persistence of deep skin ulcers, which have already been reported in association with defective expression of HLA class I molecules.
Le texte complet de cet article est disponible en PDF. This article is made possible through an educational grant from the Dermatological Division, Ortho Pharmaceutical Corporation. |
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Supported in part by the National Research Council, Roma, Italy. |
Vol 35 - N° 5P2
P. 814-818 - novembre 1996 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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