title: The spectrum of nephrocutaneous diseases and associations: Genetic causes of nephrocutaneous disease.
abstract: There are a significant number of diseases and treatment considerations of considerable importance relating to the skin and renal systems. This emphasizes the need for dermatologists in practice or in clinical training to be aware of these associations. Part I of this 2-part continuing medical education article reviews the genetic syndromes with both renal and cutaneous involvement that are most important for the dermatologist to be able to identify, manage, and appropriately refer to nephrology colleagues. Part II reviews the inflammatory syndromes with relevant renal manifestations and therapeutic agents commonly used by dermatologists that have drug-induced effects on or require close consideration of renal function. In addition, we will likewise review therapeutic agents commonly used by nephrologists that have drug-induced effects on the skin that dermatologists are likely to encounter in clinical practice. In both parts of this continuing medical education article, we discuss diagnosis, management, and appropriate referral to our nephrology colleagues in the context of each nephrocutaneous association. There are a significant number of dermatoses associated with renal abnormalities and disease, emphasizing the need for dermatologists to be keenly aware of their presence in order to avoid overlooking important skin conditions with potentially devastating renal complications. This review discusses important nephrocutaneous disease associations with recommendations for the appropriate urgency of referral to nephrology colleagues for diagnosis, surveillance, and early management of potential renal sequelae.
authors.affiliation: Department of Dermatology, Baylor University Medical Center, Dallas, Texas.
authors.forename: Jay
authors.initials: J
authors.lastname: Wofford
authors.affiliation: Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts.
authors.forename: Andrew Z
authors.initials: AZ
authors.lastname: Fenves
authors.affiliation: Department of Dermatology, University of Louisville, Louisville, Kentucky.
authors.forename: J Mark
authors.initials: JM
authors.lastname: Jackson
authors.affiliation: Department of Dermatology, Massachusetts General Hospital, Boston, Massachusetts.
authors.forename: Alexa B
authors.initials: AB
authors.lastname: Kimball
authors.affiliation: Department of Dermatology, Baylor University Medical Center, Dallas, Texas. Electronic address: amderm@gmail.com.
authors.forename: Alan
authors.initials: A
authors.lastname: Menter
mesh_terms: D001506:Beckwith-Wiedemann Syndrome / Q000150:complications / Q000235:genetics / Q000628:therapy; D058249:Birt-Hogg-Dube Syndrome / Q000150:complications / Q000235:genetics / Q000628:therapy; D000795:Fabry Disease / Q000150:complications / Q000235:genetics / Q000628:therapy; D030342:Genetic Diseases, Inborn / Q000235:genetics* / Q000628:therapy; D006223:Hamartoma Syndrome, Multiple / Q000150:complications / Q000235:genetics / Q000628:therapy; D006801:Humans; D007674:Kidney Diseases / Q000235:genetics*; D018231:Leiomyomatosis / Q000150:complications / Q000235:genetics* / Q000628:therapy; D009154:Mutation; D009261:Nail-Patella Syndrome / Q000150:complications / Q000235:genetics / Q000628:therapy; D009386:Neoplastic Syndromes, Hereditary; D009456:Neurofibromatosis 1 / Q000150:complications / Q000235:genetics / Q000628:therapy; D012871:Skin Diseases / Q000235:genetics*; D012878:Skin Neoplasms / Q000150:complications / Q000235:genetics* / Q000628:therapy; D014402:Tuberous Sclerosis / Q000150:complications / Q000235:genetics / Q000628:therapy; D014424:Turner Syndrome / Q000150:complications / Q000235:genetics / Q000628:therapy; D014594:Uterine Neoplasms / Q000150:complications / Q000235:genetics* / Q000628:therapy; D006623:von Hippel-Lindau Disease / Q000150:complications / Q000235:genetics* / Q000628:therapy
keywords: autoimmune; dermatology; genetic; genodermatoses; inflammatory; nephrocutaneous; nephrology