Treatment of multifactorial anaemia in adults with severe epidermolysis bullosa using intravenous ferric carboxymaltose: a single institution, observational, retrospective study

https://doi.org/10.1093/bjd/ljac075 Dear Editor, Anaemia in epidermolysis bullosa (EB) is largely multifactorial and is a common extracutaneous complication in severe subtypes. Left untreated, anaemia can have a significant impact on patients’ quality of life leading to fatigability, breathlessness and impaired wound healing. Oral iron supplementation may cause gastrointestinal side-­effects and has variable absorption.1,2 Occasionally, patients require frequent blood transfusions to maintain an acceptable haemoglobin (Hb) level. The consequences of unresolved anaemia in patients with EB call for early diagnosis and better maintenance treatment to improve outcomes. Consensus guidelines for the management of anaemia in EB are currently being developed; however, there are few studies concerning prevalence, course and management of anaemia in EB. Anaemia is more prevalent among patients with severe subtypes of EB for whom early monitoring of Hb levels is recommended, as anaemia can develop from the second year of life.3,4 Replacement of iron enterally or intravenously is generally recommended when Hb levels fall to 80–100 g L−1.5 There is a paucity of evidence regarding the optimum target Hb concentration in patients with severe EB, although it has been postulated that Hb levels should be optimized above 80 g L−1.6 In addition, Hb levels below 100 g L−1 cause impaired wound healing in individuals with chronic skin ulcers, owing to decreased tissue oxygenation.7 The aim of our observational, retrospective, single institution study was to investigate the efficacy and safety of periodic intravenous (IV) ferric carboxymaltose (FCM) in maintaining Hb levels ≥ 100 g L−1 in patients with severe EB in comparison with iron sucrose infusion.