title: Skin manifestations of primary immune deficiency.
abstract: Cutaneous manifestations are common in primary immune deficiency diseases, affecting between 40 % and 70 % of patients with diagnosed primary immune deficiency. Skin infections characterize many primary immune deficiencies, but there are also frequent noninfectious cutaneous manifestations seen in many of these disorders, including eczematous lesions, erythroderma, cutaneous granulomas, dysplasia of skin, hair, and nails, autoimmune conditions, and frank vasculitis. For the patient with suspected primary immunodeficiency, much can be inferred by evaluating the presenting cutaneous findings, including various infectious susceptibilities, presence of atopy, and evidence of impaired or overactive inflammatory response. The skin manifestations of primary immune deficiency diseases are often early or heralding findings of the underlying immunologic disease. Therefore, awareness of associations between skin findings and immune deficiency may aide in the early detection and treatment of serious or life-threatening immunologic defects. This review summarizes the common skin manifestations of primary immune deficiency diseases and provides the reader with a differential diagnosis of primary immune defects to consider for the most common skin manifestations.
authors.affiliation: Division of Allergy, Immunology and Pediatric Rheumatology, Department of Pediatrics, Women and Children's Hospital of Buffalo, SUNY Buffalo, School of Medicine and Biomedical Sciences, 219 Bryant Street, Buffalo, NY, 14222, USA, hkm@buffalo.edu.
authors.forename: Heather
authors.initials: H
authors.lastname: Lehman
mesh_terms: D000818:Animals; D015551:Autoimmunity; D003937:Diagnosis, Differential; D042241:Early Diagnosis; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000175:diagnosis*; D012867:Skin / Q000276:immunology / Q000473:pathology*; D012871:Skin Diseases / Q000175:diagnosis* / Q000209:etiology