Introduction: Scleromyxedema is a rare primary cutaneous mucinosis with a chronic and progressive course and possible severe systemic complications. There is no consensus on the optimal treatment for this potentially fatal disease because of the rarity of the disorder with limited number of case reports along with the lack of randomized controlled trials and incomplete etiopathogenetic understanding of the disease. Areas covered: A systematic review of the literature concerning the treatment of scleromyxedema has been performed using PubMed database, to evaluate the past and current treatments and future perspectives. Expert opinion: Treatment of scleromyxedema is challenging. Based on literature data and on the authors' experience, they recommend the use of high dose immunoglobulin as a first-line therapy. Thalidomide and/or systemic steroids are considered the second choice of treatment more often in combination with high dose immunoglobulin than as monotherapy. Promising future treatments are bortezomib and autologous stem cell transplant, but their efficacy has to be investigated.
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