Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2022 Jul;47(7):1307-1313.
doi: 10.1111/ced.15156. Epub 2022 May 25.

Establishing an appropriate level of vitamin D supplementation in paediatric patients with recessive dystrophic epidermolysis bullosa

Natalie Yerlett  1 , Antonia Loizou  1 , Maria Bageta  1 , Gabriela Petrof  1 , Anna E Martinez  1
Affiliations

Establishing an appropriate level of vitamin D supplementation in paediatric patients with recessive dystrophic epidermolysis bullosa

Natalie Yerlett et al. Clin Exp Dermatol. 2022 Jul.

Abstract

Background: Paediatric patients with recessive dystrophic epidermolysis bullosa (RDEB) are at risk of vitamin D deficiency, owing to lack of sunlight from reduced mobility and having large areas of skin being covered with dressings, and to impaired nutritional intake and status.

Aim: To establish an appropriate level of vitamin D supplementation in paediatric patients with RDEB.

Methods: Patients with RDEB attending the EB tertiary multidisciplinary team clinic were enrolled. Serum levels of total 25(OH)D were retrospectively recorded for the study period 2012-2018. Data from clinical records on supplements, bone mineral density (BMD) Z scores, compliance, and use of enteral feeds and/or formula were also recorded.

Results: In total, 24 patients met the inclusion criteria: 20 with severe RDEB, 3 with RDEB inversa and 1 with intermediate RDEB. Of the 24 patients, 21 (88%) were advised to take a vitamin D3 supplement in line with Department of Health Guidelines (UK), with the remaining 3 patients receiving sufficient intake from formula or enteral feeds. Thirteen of the 24 (54%) had vitamin D deficiency or insufficiency despite advice to supplement; 9 of these 13 (69%) subsequently started or increased the dosage of vitamin D supplements and levels became sufficient (> 50 nmol/L), while the remaining 4 patients (31%) continued to have persistent insufficient levels due to noncompliance with supplements. Reasons for noncompliance were palatability, cost and forgetting to take the tablets. The dose required to maintain sufficient serum levels increased with age, up to 300% of the reference nutrient intake (RNI).

Conclusion: All patients with RDEB require a supplement or a formula or enteral/sip feed containing vitamin D to maintain sufficient serum vitamin D. The dose required increases with age and can be up to three times higher than the RNI for the normal population. Compliance may improve using a once-weekly loading dose of vitamin D3. Vitamin D deficiency was not solely causative of a low BMD Z score.

PubMed Disclaimer

References

    1. Smith TJ, Tripkovic L, Lanham-New SA, Hart KH. Vitamin D in adolescence: evidence-based dietary requirements and implications for public health policy. Proc Nutr Soc 2018; 77: 292-301.
    1. National Institute for Health and Care Excellence. Vitamin D: supplement use in specific population groups. Public health guideline [PH56], 2014. Last updated 30 August 2017. Available at: https://www.nice.org.uk/guidance/ph56 (accessed 14 August 2021).
    1. Hubbard L, Mayre-Chilton K. Retrospective longitudinal study of osteoporosis in adults with recessive dystrophic epidermolysis bullosa. Clin Case Rep 2019; 7: 58-63.
    1. Agarwal S, Singh S, Kumar R, Sehra R. Vitamin D: a modulator of allergic rhinitis. Indian J Otolaryngol Head Neck Surg 2019; 71(Suppl): 2225-30.
    1. Hollams E. Vitamin D and atopy and asthma phenotypes in children. Curr Opin Allergy Clin Immunol 2012; 12: 228-34.

MeSH terms