Common dermatologic manifestations of primary immune deficiencies.

The skin is the largest organ of our body; it consists of the epidermis, dermis, hair follicles, sweat glands, blood vessels, and connective tissue matrix. Its main function is to act as a barrier to the outside world and protect us from infections. Any component of the skin is subject to insults from the environment and/or from within the body. Primary immune deficiency patients present with recurrent or prolonged infections not frequently seen in healthy individuals. Oftentimes, these infections involve the skin. Primary immune deficiency may also present with noninfectious cutaneous signs, such as eczema; erythroderma; granulomas; dysplasia of the skin, hair, nails, or teeth; pigmentary changes; angioedema; urticaria; vasculitis; or autoimmune skin disease due to immune dysregulation. Prompt recognition of the underlying diagnosis and initiation of treatment decrease morbidity. This review provides the reader with an up-to-date summary of the common dermatologic manifestations of primary immune deficiency diseases.

Update on the use of biologics in vasculitides.

Vasculitides are disease in which injury to blood vessels leads to various degrees and types of organ dysfunction. They are subdivided into different groups dependent on the size of blood vessels involved as well as other particular clinical features. Therapy is dependent upon the size of the blood vessels involved and the nature of the disease. For medium and large vessel vasculitides, glucocorticoids, cyclophosphamide, azathioprine and methotrexate have been the mainstay for induction and maintenance of remission. Because of potential for side effects from standard therapies, various biologic agents have been evaluated in the treatment of ANCA positive vasculitis (AAV), cryoglobulinemic vasculitis, Behcet's disease and Takayasu's Arteritis (TAK). In this article, we present a review on biologic agents used in various vasculitides.

Update on the use of biologics in lupus.

Systemic lupus erythematosus is more than a heterogeneous autoimmune disease. It is in fact a syndrome of many different clinical genotypes and phenotypes. This review covers the most recently studied monoclonal antibodies and those currently being investigated. The key trials are summarized, including those for biologics on the market for two decades and those just approved for lupus less than two years ago. The focus will be on the following four: Rituximab, Abatacept, Belimumab, and Epratuzumab. It is a challenge to design the perfect trial and study patients with this disease as each has his or her own unique manifestations, biological markers, and underlying genetic background. However, as our understanding of the immune mechanisms involved in lupus increases; novel therapies will emerge that can be utilized for appropriately selected patients.