title: Scleromyxoedema and thrombotic thrombocytopaenic purpura: two rare conditions both responding to plasma exchange.
abstract: We report the case of a 66-year-old female who over an 18-month period developed severe, disabling scleromyxoedema with pulmonary fibrosis. Treatment with oral prednisolone and melphalan had failed to prevent disease progression. Treatment with a 5-day course of plasma exchange, intravenous cyclophosphamide (500 mg) and methyl-prednisolone (1 g on 3 consecutive days) was unfortunately followed by the development of thrombotic thrombocytopaenic purpura (TTP). After 17 extra plasma exchanges, she recovered and there has been a dramatic improvement in her skin signs. We postulate that the extra plasma exchanges which she received as a consequence of developing TTP have contributed to this result. To our knowledge, TTP has never been associated with scleromyxoedema, but it is likely to be a coincidence in this case.
authors.affiliation: St. John's Institute of Dermatology, St. Thomas' Hospital, London, UK. k.harman@umds.ac.uk
authors.forename: K E
authors.initials: KE
authors.lastname: Harman
authors.forename: I C
authors.initials: IC
authors.lastname: Abbs
authors.forename: J M
authors.initials: JM
authors.lastname: Mahood
authors.forename: M M
authors.initials: MM
authors.lastname: Black
mesh_terms: D000368:Aged; D005260:Female; D006801:Humans; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications* / Q000473:pathology / Q000628:therapy; D012595:Scleroderma, Systemic / Q000150:complications* / Q000473:pathology / Q000628:therapy