Peripheral Blood Mononuclear Cell Subsets in Patients With Severe Inherited Forms of Epidermolysis Bullosa

Abstract

Alterations in monocyte and lymphocyte subsets known to affect host immune response were observed in patients with severe forms of EB. Quantitative changes relative to controls included decreased total numbers of T cells with greater decreases in helper cells, decreased NK cells, and a diminished number of interleukin 2 receptors. Such changes have been associated previously with a lower resistance to infections and to neoplasia. The changes in subsets correlated with the severity of the cutaneous and extracutaneous disease in the patients with EB.