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.

Nephrogenic fibrosing dermopathy and calciphylaxis with pseudoxanthoma elasticum‐like changes

“…Foci of dermal calcification, often in close approximation to dermal elastic fibers, have also been reported 3–10 . The clinical and histopathologic features of NFD, in particular the pattern of fibrosis, were initially thought to be reminiscent of scleromyxedema 10–12 . Most, though not all, reported cases have occurred in patients with dialysis‐dependent renal failure 5 .…”

Coma associated with scleromyxoedema and interferon alfa therapy. Full recovery after steroids and cyclophosphamide combined with plasmapheresis

“…In the remainder, it regressed over 2–20 days without sequelae. Three patients including ours developed acute renal failure 4,6 . The patient of Kantor et al 4 .…”

“…Neurological symptoms affect 15% of the patients, including memory loss, confusion, vertigo, hallucinations and depression, acute psychosis, seizures, syncope, dysarthria, aphasia and ascending paralysis 1 . At least 19 cases of coma (including ours) have been reported as a manifestation of SCL encephalopathy 1–8 . Comatose patients were aged 32–66 years, and males predominated (12 : 7).…”

“…A clear pathogenetic mechanism for renal failure in our patient was not established. Five of the patients who recovered, including ours, were treated with plasmapheresis 6 …”

“…The patient of Kantor et al 4 . had scleroderma renal disease while in that of Harman et al 6 . both coma and renal complications were attributed to thrombotic thrombocytopenic purpura.…”