Dermato-neuro syndrome in a case of scleromyxedema

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.

Monoclonal gammopathy of undetermined significance-associated scleromyxoedema

“…It has been proposed that an increased blood viscosity with impaired microcirculation along with an increase in serum factor VIII, and von Willebrand factor caused by paraproteinemia may result in encephalopathy-like symptoms. [ 5 ] In our case, the central nervous system involvement may be attributed to this mechanism, however, it could not be investigated further owing to the relatively short duration of the symptoms. Further, a cerebrospinal fluid examination was desirable, but could not be conducted as the patient gave a negative consent.…”

Scleromyxedema in a 21 year old female patient with acute lymphoblastic leukemia: a case report

“…These included, but not limited to, monoclonal gammopathy, lymphoma, advanced gastric cancer, and very rarely, acute leukemia [ 6 , 9 ]. Scleromyxedema can lead to disabling systemic manifestations, these include: dysphagia, myopathy [ 13 ], cardiomyopathy [ 6 ], central nervous system involvement, including: intractable fever, convulsion, and coma [ 14 ].…”

Granulomatöses Skleromyxödem mit dermato-neurologischem Syndrom – Vorstellung einer seltenen Erkrankung mit schwerer Komplikation

“…Das DNS ist definiert als die Kombination Grippe-ähnlicher Symptome, hohem Fieber, tonisch-klonischer Krämpfe und Koma im klinischen Kontext eines Skleromyxödems [10]. Letale Verläufe des Syndroms wurden beschrieben [11]. Fachspezifisch ist das DNS von weiteren neurologischen Assoziationen des SME wie Enzephalopathie, akuten Psychosen, kognitiven Erkrankungen, peripherer Neuropathie und dem Karpaltunnelsyndrom abzugrenzen, welche in 10 -15 % der Patienten mit SME beobachtet werden können [12,13].…”