title: Scleredema. A multicentre study of characteristics, comorbidities, course and therapy in 44 patients.
abstract: BACKGROUND
  The prognostic and therapeutic features of scleredema are poorly documented.
  
  
  OBJECTIVE
  To describe the characteristics of patients with scleredema regarding demographics, clinical characteristics, comorbidities, therapeutic interventions and course.
  
  
  METHODS
  We conducted a retrospective multicentre study.
  
  
  RESULTS
  We identified 44 patients (26 men).The mean age at diagnosis was 53.8 years. The most common associated disorders were endocrine/metabolic diseases including 30 patients suffering from diabetes, mostly type 2 diabetes. Monoclonal gammopathies were confirmed in five cases. A preceding respiratory tract infection was not a feature. Treatments with different combination or sequential modalities were used with variable results. Phototherapy (UVA1 or PUVA) was the treatment associated with higher, although partial response. Systemic corticosteroids and immunosuppressive drugs were reserved to patients with severe disease in whom phototherapy had failed or for patients with multiple myeloma. Forty-one patients were followed up (mean period: 32.2 months).Thirty-nine patients are alive, 30 with and 9 without skin disease. Two patients died of cardiovascular complications due to myeloma and severe diabetes.
  
  
  CONCLUSIONS
  Scleredema is a chronic debilitating disease associated with diabetes and metabolic syndrome, unresponsive to various treatments but not necessarily a life-threatening condition. Although there is no definitive treatment, phototherapy should be attempted first. Treatment of primary disease including strict glycaemic control combined with physical therapy should be also employed.
authors: IRCSS-AOU S. Martino-IST, Department of Health Sciences, DISSAL, Section of Dermatology, University of Genoa, Genoa, Italy.
authors: F
authors: F
authors: Rongioletti
authors: IRCSS-AOU S. Martino-IST, Department of Health Sciences, DISSAL, Section of Dermatology, University of Genoa, Genoa, Italy.
authors: F
authors: F
authors: Kaiser
authors: IRCSS-AOU S. Martino-IST, Department of Health Sciences, DISSAL, Section of Dermatology, University of Genoa, Genoa, Italy.|Section of Dermatology, University Hospital of Saint-Etienne, Saint-Etienne, France.
authors: E
authors: E
authors: Cinotti
authors: Department of Dermatology, University Hospital, Munster, Germany.
authors: D
authors: D
authors: Metze
authors: Department of Pathology, Hôpital Saint Louis, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors: M
authors: M
authors: Battistella
authors: Department of Dermatology, University Hospital Spedali Civili, Brescia, Italy.
authors: P G
authors: PG
authors: Calzavara-Pinton
authors: Clinic of Dermatology, Medical Faculty University "Ss Cyril and Methodius", Skopje, Republic of Macedonia.
authors: K
authors: K
authors: Damevska
authors: Department of Medicine, Section of Dermatology and Venereology, University of Verona, Verona, Italy.
authors: G
authors: G
authors: Girolomoni
authors: Department of Dermatology, CHU-Saint Pierre, Free University of Brussels, Brussels, Belgium.
authors: J
authors: J
authors: André
authors: Section of Dermatology, University Hospital of Saint-Etienne, Saint-Etienne, France.
authors: J-L
authors: JL
authors: Perrot
authors: Kempf and Pfaltz, Histological Diagnostics, and Dept. of Dermatology, University Hospital Zürich, Zürich, Switzerland.
authors: W
authors: W
authors: Kempf
authors: Service de Dermatopathologie, Paris, France.
authors: B
authors: B
authors: Cavelier-Balloy
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D015897:Comorbidity; D003924:Diabetes Mellitus, Type 2 / Q000453:epidemiology*; D050171:Dyslipidemias / Q000453:epidemiology; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009765:Obesity / Q000453:epidemiology; D011701:PUVA Therapy; D010265:Paraproteinemias / Q000453:epidemiology*; D012189:Retrospective Studies; D012592:Scleredema Adultorum / Q000188:drug therapy* / Q000453:epidemiology*; D015996:Survival Rate; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents