Nutrition Management of Patients with Epidermolysis Bullosa

• Published in: Journal of the American Dietetic Association 1995-05, Vol.95 (5), p.575-579
• Main Author: BIRGE, KRISTINE
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Avitaminosis - drug therapy; Avitaminosis - etiology; Biological and medical sciences; Bullous diseases of the skin; Child; Child, Preschool; children; Constipation - etiology; Constipation - therapy; deficiency diseases; Deglutition Disorders - etiology; Dermatology; Diet therapy; enfant; enfermedades carenciales; enfermedades de la piel; Epidermolysis bullosa; Epidermolysis Bullosa - complications; Epidermolysis Bullosa - diet therapy; estado nutricional; etat nutritionnel; Female; genetic disorders; Humans; Infant; maladie de carence; maladie de la peau; Male; Mastication; Medical disorders; Medical sciences; Middle Aged; ninos; Nutrition; nutritional status; peso; poids; Protein-Energy Malnutrition - etiology; Protein-Energy Malnutrition - prevention & control; riesgo; risk; risque; Skin; Skin diseases; trastornos geneticos; trouble genetique; weight
• ISSN: 0002-8223; 2212-2672; 1878-3570; 2212-2680
• DOI: 10.1016/S0002-8223(95)00157-3

Epiderrnolysis bullosa (EB), a heterogeneous group of rare, inherited disorders, is manifested by recurrent blistering of the skin induced by the slightest trauma. Little information exists regarding the nutrition management of patients with EB. This study presents information on growth, identifies potential nutrition problems, and provides guidelines for nutrition management of persons with EB. Eighty patients attending a dermatology clinic for EB patients are described. Severity of disease ranged from mild blistering of the knees, elbows, and feet to extensive blistering and scarring of the skin and entire gastrointestinal tract. Of the 18 children with EB simplex, which is a mild form of the disease, 4 (22%) were at nutritional risk. None of the 13 adults with EB simplex were underweight and 8 (62%) were overweight. Of the patients with the more severe forms of EB, 27 of the 35 (77%) children with dystrophic EB and 4 of the 7 (57%) children with junctional EB were at risk for malnutrition. Of the 7 adults with dystrophic EB, 6 (86%) were underweight. Common nutrition problems included protein-energy malnutrition, chewing and swallowing problems, constipation, anemia, and vitamin/mineral deficiencies. When nutrition care protocols address these problems, growth, development, and nutritional status can improve. For those with severe nutrition problems, gastrostomy feeding or similar nutrition therapies should be considered. J Am Diet Assoc. 1995; 95:575-579.