Defective expression of HLA class I and CD1a molecules in boy with Marfan-like phenotype and deep skin ulcers
A Plebani, V Monafo, R Cattaneo, G Carella… - Journal of the American …, 1996 - Elsevier
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 …
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 …