Defective expression of HLA class I and CD1a molecules in boy with Marfan-like phenotype and deep skin ulcers.

doi:10.1016/s0190-9622(96)90091-2

Article

Defective expression of HLA class I and CD1a molecules in boy with Marfan-like phenotype and deep skin ulcers.

Journal of the American Academy of Dermatology - 1 Nov 1996

Plebani A, Monafo V, Cattaneo R, Carella G, Brugnoni D, Facchetti F, Battocchio S, Meini A, Notarangelo L D, Duse M, Ugazio A G

Abstract excerpt

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...

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Defective expression of HLA class I and CD1a molecules in boy with Marfan-like phenotype and deep skin ulcers.

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