Dermato-neuro syndrome in a case of scleromyxedema


Savran Y., AKARSU S.

EUROPEAN JOURNAL OF RHEUMATOLOGY, vol.2, no.4, pp.160-162, 2015 (ESCI, TRDizin)

  • Publication Type: Article / Article
  • Volume: 2 Issue: 4
  • Publication Date: 2015
  • Doi Number: 10.5152/eurjrheum.2015.0116
  • Journal Name: EUROPEAN JOURNAL OF RHEUMATOLOGY
  • Journal Indexes: Emerging Sources Citation Index (ESCI), TR DİZİN (ULAKBİM)
  • Page Numbers: pp.160-162
  • Keywords: Scleromyxedema, dermato-neuro syndrome, connective tissue
  • Dokuz Eylül University Affiliated: Yes

Abstract

Scleromyxedema is an uncommon connective tissue disease characterized by mucin deposits, fibrosis, and proliferation of fibroblasts in the dermis. Although it shares similar sclerodermoid features, it is a different clinical entity than scleroderma. A monoclonal gammopathy is almost always present; however, progression to multiple myeloma is rare. It may have many systemic manifestations, of which the most notable being the dermato-neuro syndrome because of its rarity and potential fatal outcome. We present a case of a 50-year-old woman with scleromyxedema in whom the dermato-neuro syndrome developed.