Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part II. Other organs.
It is well known, primarily via case reports and limited case series, that nonepithelial tissues may become injured in patients with epidermolysis bullosa. Only recently, however, have there been data generated from large, well characterized cohorts. Our objective is to provide dermatologists with a comprehensive review of each of these major extracutaneous complications, with a summary of the pertinent literature and evidence-based recommendations for surveillance, evaluation, and management. Some epidermolysis bullosa subtypes are at risk for severe injury of the bone marrow, musculoskeletal system, heart, kidney, and teeth, and for the development of squamous cell carcinoma, basal cell carcinoma, or malignant melanoma. If untreated, significant morbidity or mortality may result.
Fine
Jo-David
JD
The National Epidermolysis Bullosa Registry, and Department of Medicine (Dermatology), Vanderbilt University School of Medicine, Nashville, Tennessee, USA. Jo-David.Fine@vanderbilt.edu
Mellerio
Jemima E
JE
D002294:Carcinoma, Squamous Cell / Q000150:complications; D003880:Dermatology; D004502:Education, Medical, Continuing; D004820:Epidermolysis Bullosa / Q000150:complications / Q000473:pathology / Q000628:therapy; D006331:Heart Diseases / Q000209:etiology*; D006801:Humans; D009059:Mouth Diseases / Q000209:etiology; D009140:Musculoskeletal Diseases / Q000209:etiology*; D009422:Nervous System Diseases / Q000209:etiology*; D012878:Skin Neoplasms / Q000150:complications