This protocol applies to scleredema occurring in the context of an associated haematological disease. The presentation is symmetrical, woody, non-pitting skin induration of the neck and upper trunk, characteristically sparing the fingers.
Type 2 scleredema is associated with haematological diseases including paraproteinaemia — such as monoclonal gammopathy — as well as multiple myeloma and amyloidosis. Identifying the underlying haematological condition is central to directing management.
Management draws on a range of advanced-line options — encompassing both systemic agents and physical modalities — chosen on an individualised risk–benefit basis. The full protocol sets out the complete framework for selection.
Type 2 scleroedema is associated with haematological diseases like paraproteinaemia including monoclonal gammopathy, multiple myeloma and amyloidosis.
If methotrexate fails or is contraindicated, based on a risk–benefit approach, the following alternative treatments can be proposed: glucocorticoids, systemic or intralesional, cyclosporine, prostaglandin E1, intravenous immunoglobulins, high-dose penicillin, factor XIII infusion, cyclophosphamide, tranilast, thalidomide, bortezomib, radiotherapy, extracorporeal shock wave therapy, electron-beam radiotherapy and extracorporeal photopheresis.
DOI: 10.1111/jdv.19937
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