Defective expression of HLA class I and CD1a molecules in boy with Marfan-like phenotype and deep skin ulcers.
J Am Acad Dermatol
; 35(5 Pt 2): 814-8, 1996 Nov.
Article
em En
| MEDLINE
| ID: mdl-8912593
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.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Antígenos CD1
/
Antígenos HLA
/
Úlcera da Perna
/
Síndrome de Marfan
Limite:
Adolescent
/
Humans
/
Male
Idioma:
En
Revista:
J Am Acad Dermatol
Ano de publicação:
1996
Tipo de documento:
Article