GENETIC IMMUNODEFICIENCIES - CUTANEOUS MANIFESTATIONS AND RECENT PROGRESS

In recent years, remarkable progress has been made in elucidating the pathophysiology of genetic immunodeficiency disorders. Dermatologic ma nifestations are prominent in these conditions; because of advances in diagnosis and therapy, patients are living longer, increasing the lik elihood that dermatologists will encounter patients with these disease s. The genes of many of these disorders have been cloned, including ch ronic granulomatous disease, X-linked immunodeficiencies, and myeloper oxidase deficiency. Understanding the regulation and function of these genes will not only affect patients with these rare disorders, but ma y provide an insight into common dermatologic conditions, such as ecze ma and cutaneous infection. Diagnosis, dermatologic manifestations, an d therapy are discussed.