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Scleromyxoedema and thrombotic thrombocytopaenic purpura: two rare conditions both responding to plasma exchange
Author(s) -
Harman Karen E.,
Abbs Ian C.,
Mahood John M.,
Black Martin M.
Publication year - 1998
Publication title -
journal of the european academy of dermatology and venereology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.655
H-Index - 107
eISSN - 1468-3083
pISSN - 0926-9959
DOI - 10.1111/j.1468-3083.1998.tb00956.x
Subject(s) - medicine , prednisolone , purpura (gastropod) , cyclophosphamide , plasmapheresis , thrombotic thrombocytopenic purpura , melphalan , dermatology , gastroenterology , surgery , chemotherapy , immunology , platelet , ecology , antibody , biology
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.