Osteopenia and osteoporosis in epidermolysis bullosa

Abstract

Patients with the more severe forms of epidermolysis bullosa (EB) are at risk of developing osteopenia, osteoporosis and fractures. The cause is likely to be multifactorial and includes reduced mobility, a generally proinflammatory state, poor nutrition and hormonal factors. Monitoring this group with dual energy X-ray absorptiometry (DEXA) scans and plain radiographs is necessary to detect these changes. Data are lacking about the optimal approach to managing poor bone health in EB, although it seems that encouraging mobility, supplementation of calcium and vitamin D where necessary, with the addition of a bisphosphonate when there is evidence of fractures, may be helpful.