title: Scleromyxedema: a multicenter study of characteristics, comorbidities, course, and therapy in 30 patients.
abstract: BACKGROUND
  Scleromyxedema is associated with a monoclonal gammopathy and other comorbidities. Its prognostic and therapeutic features are poorly documented because most reports deal with single cases or small series.
  
  
  OBJECTIVE
  We sought to describe the characteristics of patients with scleromyxedema regarding demographics, clinical characteristics, comorbidities, therapeutic interventions, and course.
  
  
  METHODS
  We conducted a retrospective and prospective multicenter study.
  
  
  RESULTS
  We identified 30 patients with scleromyxedema (17 men and 13 women). The mean age at diagnosis was 59 years. The mean delay between disease onset and diagnosis was 9 months. Monoclonal gammopathy was detected in 27 patients. Extracutaneous manifestations were present in 19 patients including neurologic (30%), rheumatologic (23.3%), and cardiac (20%) manifestations. Two patients developed hematologic malignancies. The most common therapies included oral steroids and intravenous immunoglobulins. Although corticosteroids were ineffective, intravenous immunoglobulins (alone or in combination with other drugs) induced complete remission in 4 and partial remission in 9 patients with a mean treatment duration of 2 years. In all, 21 patients were followed up for a mean period of 33.5 months, at which time 16 patients were alive, 12 with and 4 without skin disease. Five patients died: 2 with dermatoneuro syndrome and 1 each with myeloid leukemia, Hodgkin lymphoma, and myocardial insufficiency.
  
  
  CONCLUSIONS
  This is mainly a retrospective study.
  
  
  CONCLUSIONS
  Our study confirms that scleromyxedema is a chronic and unpredictable disease with severe systemic manifestations leading to a guarded prognosis. There is no specific definitive treatment. Our data support the contention that intravenous immunoglobulin is a relatively effective and safe treatment. The response is not permanent and maintenance infusions are required.
authors: Section of Dermatology, Dipartimento di Scienze della Salute, University of Genoa and S. Martino University Hospital, Genoa, Italy. franco.rongioletti@unige.it
authors: Franco
authors: F
authors: Rongioletti
authors: Giulia
authors: G
authors: Merlo
authors: Elisa
authors: E
authors: Cinotti
authors: Valentina
authors: V
authors: Fausti
authors: Emanuele
authors: E
authors: Cozzani
authors: Bernard
authors: B
authors: Cribier
authors: Dieter
authors: D
authors: Metze
authors: Eduardo
authors: E
authors: Calonje
authors: Jean
authors: J
authors: Kanitakis
authors: Werner
authors: W
authors: Kempf
authors: Catherine M
authors: CM
authors: Stefanato
authors: Eduardo
authors: E
authors: Marinho
authors: Aurora
authors: A
authors: Parodi
mesh_terms: D015897:Comorbidity; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000453:epidemiology; D011379:Prognosis; D011446:Prospective Studies; D012074:Remission Induction; D053718:Scleromyxedema / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology; D013308:Streptonigrin; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; C079133:retrostatin; D013308:Streptonigrin