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

Alessandro Plebani, Virginia Monafo, Roberto Cattaneo, Graziella Carella, Duilio Brugnoni, Fabio Facchetti, Simonetta Battocchio, Antonella Meini, Luigi D. Notarangelo, Marzia Duse, Alberto G. Ugazio

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)814-818
Number of pages5
JournalJournal of the American Academy of Dermatology
Volume35
Issue number5 II
Publication statusPublished - Nov 1996

ASJC Scopus subject areas

  • Dermatology

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