British Journal of Dermatology
British Journal of Dermatology
5 years ago

Osteoporosis in erythropoietic protoporphyria patients

M. Langeveld, H. Koole-Lesuis, M. Biewenga, E.C.H. Friesema, R.H.S. Matawlie, J.G. Langendonk, J.H.P. Wilson
Background Erythropoietic protoporphyria is a rare metabolic disease with painful photosensitivity due to protoporphyrin IX accumulation, resulting in lifelong light avoidance behaviour. Objectives The aim of this study was to evaluate bone mineral density and known osteoporosis risk factors in erythropoietic protoporphyria patients. Methods In this cross-sectional study all erythropoietic protoporphyria patients attending the Erasmus MC outpatient clinic that had undergone bone mineral density measurements were included. Plasma 25 OH-vitamin D, alkaline phosphatase, parathyroid hormone and total protoporphyrin IX levels were measured and information on life style, sun light exposure and a bone relevant physical exercise index (BPAQ score) was obtained using questionnaires. Bone mineral density scores and the prevalence of osteopenia and osteoporosis in the erythropoietic protoporphyria population were compared to a reference population. Results Twenty-three female and twenty-one male erythropoietic protoporphyria patients with a mean age of 37.6 years were included. The mean SD of the T-scores were -1.12 for the lumbar spine and -0.82 for the femoral neck (p<0.001 for both). Osteopenia was present in 35.9% and osteoporosis in 23.1%. Based on the reference population the expected prevalence was 15% and 0.6%, respectively. The prevalence of vitamin D deficiency was 50% (defined as a 25-OH vitamin D level under 50 nmol/l). The mean self-reported BPAQ score was 19.4 units (reference 19-24 units). Multiple linear regression analysis showed a significant influence of vitamin D deficiency and bone relevant physical exercise score on bone mineral density in erythropoietic protoporphyria patients. Conclusions The prevalence of osteoporosis and osteopenia is greatly increased in erythropoietic protoporphyria patients. Factors that significantly correlate with low bone mineral density in this population are alkaline phosphatase (related to vitamin D deficiency) and the amount of weight bearing exercise. This article is protected by copyright. All rights reserved.

Publisher URL: http://onlinelibrary.wiley.com/resolve/doi

DOI: 10.1111/bjd.15893

You might also like
Discover & Discuss Important Research

Keeping up-to-date with research can feel impossible, with papers being published faster than you'll ever be able to read them. That's where Researcher comes in: we're simplifying discovery and making important discussions happen. With over 19,000 sources, including peer-reviewed journals, preprints, blogs, universities, podcasts and Live events across 10 research areas, you'll never miss what's important to you. It's like social media, but better. Oh, and we should mention - it's free.

  • Download from Google Play
  • Download from App Store
  • Download from AppInChina

Researcher displays publicly available abstracts and doesn’t host any full article content. If the content is open access, we will direct clicks from the abstracts to the publisher website and display the PDF copy on our platform. Clicks to view the full text will be directed to the publisher website, where only users with subscriptions or access through their institution are able to view the full article.