Plasmapheresis in a Patient With Scleromyxedema

Abstract

We describe a patient with progressive scleromyxedema who failed to respond to previously described treatment modalities. A trial of plasmapheresis was initiated, but after an initial promising response, his disease continued to progress. The skin involvement appeared to progress even more rapidly after the plasmapheresis was discontinued.

Treatment of scleromyxoedema with hydroxychloroquine

“…Although scleromyxoedema was first described almost 50 years ago in 1954 [1], pathogenesis is still unknown. Paraproteinaemia seems to be pivotal but therapeutic efforts aiming at the removal of immunoglobulins fail to influence skin disease [17]. This was confirmed in Patient 1, who showed no response to 20 cycles of plasmapheresis (in combination with an immunosuppressive therapy).…”

“…However, chemotherapeutic drugs reveal potentially severe side effects and should be reserved for cases with systemic symptoms when less toxic treatment options have failed [6]. Plasmapheresis [15, 17] and/or photopheresis [18] are frequently used to reduce conceivably pathogenic factors. Other treatment options for scleromyxoedema include high‐dose intravenous immunoglobulin therapy [19], whole‐body electron‐beam therapy [20], isotretinoin [9], interferon‐alpha [21], or locally applied dimethyl sulfoxide/corticosteroid combination [22].…”

Scleromyxedema: Successful treatment with IVIg and lenalidomide

“…8,9,10 Combination of thalidomide and IVIg has been used most frequently. 9,11 The recently introduced highly effective anti-plasma cell therapy such as bortezomib, thalidomide and lenalidomide has been shown to provide a higher likelihood of achieving a complete response. The favorable toxicity profile of these agents makes it a more reasonable treatment, even in patients without manifestations of multiple myeloma.…”

Scleromyxedema with monoclonal gammopathy and neurological involvement: recovery from coma after plasmapheresis?

“…Plasmapheresis may be an option for this circumstance, but not all patients show improvement. Westhein and Lookingbill reported a patient with severe longstanding skin disease with no evidence of neurological damage who was treated with plasmapheresis; the response was transient, and the skin disease progressed rapidly after the discontinuation of plasmapheresis 14 . Eventual responses to intravenous immunoglobulin 15 and stem cell transplantation 16 have been reported.…”