Vitamin and trace metal levels in recessive dystrophic epidermolysis bullosa.
BACKGROUND
In recessive dystrophic epidermolysis bullosa (RDEB), a good nutritional balance is necessary to obtain healing of the chronic wounds. However, involvement of the oral mucosa and oesophagus stenosis may be responsible for severe nutritional deficiencies.


OBJECTIVE
In order to propose an adapted nutritional management, we studied the vitamin and trace metal status of 14 RDEB patients.


METHODS
Height and weight were measured. Plasma levels of albumin, iron, ferritin, calcium, parathyroid hormone (PTH), folates, vitamins C, D, B12, A, E, B1, B6, PP and B2, zinc, selenium, carnitine and copper were measured.


RESULTS
Most patients had a significant growth retardation. We found iron, vitamin D, C, B6, PP, zinc and selenium deficiencies in 36-70% of the patients, without clinical expression, except in one case. Vitamin B1, 12, B2, A/RBP, E/lipids and carnitine were normal. The three patients with gastrostomy feeding had better growth but still a protein deficiency and sometimes vitamin C, B6, PP, zinc and carnitine deficiencies.


CONCLUSIONS
Vitamin and trace metal deficiencies are frequent in RDEB, even in patients receiving gastrostomy feeding, and often go unrecognized. Regular nutritional evaluation is necessary. Dietary advice and supplements should be given. Enteral feeding by gastrostomy should be discussed in early childhood.
Ingen-Housz-Oro
S
S
Department of Dermatology, Saint-Louis Hospital, 1 avenue C. Vellefaux, 75475 Paris Cedex 10, France. saskia.oro@ch-argenteuil.fr
Blanchet-Bardon
C
C
Vrillat
M
M
Dubertret
L
L
D000293:Adolescent; D000328:Adult; D001361:Avitaminosis / Q000209:etiology; D002648:Child; D003430:Cross-Sectional Studies; D016108:Epidermolysis Bullosa Dystrophica / Q000097:blood* / Q000150:complications / Q000235:genetics; D005260:Female; D005774:Gastrostomy; D006801:Humans; D008297:Male; D009752:Nutritional Status; D014131:Trace Elements / Q000097:blood*; D014815:Vitamins / Q000097:blood*
D014131:Trace Elements; D014815:Vitamins