Dermato-neuro syndrome in a case of scleromyxedema

Savran Y., AKARSU S.

EUROPEAN JOURNAL OF RHEUMATOLOGY, cilt.2, sa.4, ss.160-162, 2015 (ESCI) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 2 Sayı: 4
  • Basım Tarihi: 2015
  • Doi Numarası: 10.5152/eurjrheum.2015.0116
  • Dergi Adı: EUROPEAN JOURNAL OF RHEUMATOLOGY
  • Derginin Tarandığı İndeksler: Emerging Sources Citation Index (ESCI), TR DİZİN (ULAKBİM)
  • Sayfa Sayıları: ss.160-162
  • Anahtar Kelimeler: Scleromyxedema, dermato-neuro syndrome, connective tissue
  • Dokuz Eylül Üniversitesi Adresli: Evet

Özet

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.