Immunological abnormalities in massive cutaneous hyalinosis.
Immunological studies in a patient with massive cutaneous hyalinosis, a disease characterized by principally dermal and subcutaneous accumulations of monoclonal kappa light chains and a gliadin-binding mannose-rich 90 kD glycoprotein, show that the ratio of helper to suppressor T cells is decreased and the proliferative responses of the peripheral mononuclear cells to T cell and T cell-dependent B cell mitogens are depressed. High levels of circulating immune complexes were demonstrated by C1q-binding and rheumatoid factor enzyme linked immunoassays. IgM and IgA class antibodies against the hyalin components, the mannosyl-90 kD glycoprotein and type I collagen, and against keratin and gluten were present in high titres. The reactivity of mononuclear cells to phytohemagglutinin normalized and the antibody levels to hyalin proteins, keratin and gluten fell during low-dose steroid therapy. However, the concanavalin A response was not reversed, neither did the levels of circulating immune complexes and anti-intercellular substance antibodies decrease. The results demonstrate a very complex dysfunction of the immune system in massive cutaneous hyalinosis.
Maury
C P
CP
Teppo
A M
AM
D000368:Aged; D000936:Antigen-Antibody Complex / Q000032:analysis; D005260:Female; D006801:Humans; D006818:Hyalin / Q000276:immunology*; D007070:Immunoglobulin A / Q000032:analysis; D007075:Immunoglobulin M / Q000032:analysis; D007376:Interleukin-2 / Q000032:analysis; D012871:Skin Diseases / Q000276:immunology*; D013601:T-Lymphocytes / Q000276:immunology; D006377:T-Lymphocytes, Helper-Inducer / Q000276:immunology
D000936:Antigen-Antibody Complex; D007070:Immunoglobulin A; D007075:Immunoglobulin M; D007376:Interleukin-2