Is oestrogen therapy justified in the treatment of hereditary haemorrhagic telangiectasia: A biochemical evaluation

Systemic and topical oestrogen can provoke squamous metaplasia of epithelium. In Hereditary Haemorrhagic Telangiectasia (HHT) the underlying telangiectasia may be protected from trauma and epistaxis reduced. Oestrogens have been advocated but their efficacy is unclear. Recent advances have now identified two oestrogen and one progesterone receptors. The aim of this study is to analyse the sex receptor status of HHT nasal mucosa to determine if oestrogen therapy is biochemically justified. Five HHT patients (three men, two women) and eight controls (four men, four women) underwent nasal mucosa biopsy. Samples were fixed in formalin and paraffin embedded. Alpha oestrogen (ERα) and beta oestrogen (ERβ) and progesterone (PgR) receptors were identified using mouse monoclonal antibodies by the Streptavidin-biotin peroxidase method. ERβ was detected in two HHT subjects (1 M: 1F) and two control subjects. ERα and PgR was absent in HHT Subjects. This pilot study demonstrated that a subgroup of HHT patients were ERβ positive. Oestrogen therapy therefore has a potential therapeutic role on a biochemical basis in these patients. ERβ status should be determined before considering oestrogen therapy.