Is oestrogen therapy justified in the treatment of hereditary haemorrhagictelangiectasia: a biochemical evaluation

Systemic and topical oestrogen can provoke squamous metaplasia of epitheliu m. In Hereditary Haemorrhagic Telangiectasia (HHT) the underlying telangiec tasia may be protected from trauma and epistaxis reduced. Oestrogens have b een advocated but their efficacy is unclear. Recent advances have now ident ified two oestrogen and one progesterone receptors. The aim of this study i s to analyse the sex receptor status of HHT nasal mucosa to determine if oe strogen therapy is biochemically justified. Five HHT patients (three men, t wo women) and eight controls (four men, four women) underwent nasal mucosa biopsy. Samples were fixed in formalin and paraffin embedded. Alpha oestrog en (ER alpha) and beta oestrogen (ER beta) and progesterone (PgR) receptors were identified using mouse monoclonal antibodies by the Streptavidin-biot in peroxidase method. ER beta was detected in two HHT subjects (1 M: 1F) an d two control subjects. ER alpha and PgR was absent in HHT subjects. This p ilot study demonstrated that a subgroup of HHT patients were ER beta positi ve. Oestrogen therapy therefore has a potential therapeutic role on a bioch emical basis in these patients. ER beta status should be determined before considering oestrogen therapy.