title: Common dermatologic manifestations of primary immune deficiencies.
abstract: 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.
authors.affiliation: Division of Allergy/Immunology/Pediatric Rheumatology, Department of Pediatrics, Women and Children's Hospital of Buffalo, SUNY at Buffalo School of Medicine and Biomedical Sciences, 219 Bryant Street, Buffalo, NY, 14222, USA.
authors.forename: Manisha
authors.initials: M
authors.lastname: Relan
authors.forename: Heather K
authors.initials: HK
authors.lastname: Lehman
mesh_terms: D003873:Dermatitis, Exfoliative / Q000276:immunology*; D006105:Granulomatous Disease, Chronic / Q000276:immunology*; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000276:immunology*; D012867:Skin / Q000276:immunology*; D012874:Skin Diseases, Infectious / Q000276:immunology*