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Clinical Characteristics and Treatment Outcomes of Scleromyxedema: A 10-Year Retrospective Survey Publisher



Ansari A1 ; Erfani Z1 ; Daneshpazhooh M1 ; Mahmoudi L1 ; Saffarian Z1 ; Kamyab K2 ; Mahmoudi H1
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Authors Affiliations
  1. 1. Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran
  2. 2. Department of Dermatopathology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran

Source: Case Reports in Dermatology Published:2022


Abstract

Scleromyxedema is a rare but important mucinosis disorder of the skin that is presented with dermatological manifestations such as waxy papules, diffuse induration, and nondermatologic involvements like neurological and renal disorders. We report a case series of the data regarding the characteristics and treatment of 14 patients diagnosed with scleromyxedema and their follow-up. Patients entered the study based on scleromyxedema diagnosis criteria. Comorbidities were also recorded to evaluate their effect on the treatment process. Clinicopathological and laboratory findings and responses to their treatment were evaluated separately. There was a significant improvement after administering intravenous immunoglobulin (IVIG). Despite the lack of a definite treatment for this condition, the present study shows that the application of IVIG can improve both cutaneous and systemic symptoms. Younger patients, in particular, responded significantly to the use of IVIG. More studies are required to investigate the potential efficacy of IVIG in the treatment of scleromyxedema. © 2022 The Author(s). Published by S. Karger AG, Basel.
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