F Rongioletti1, F Kaiser1, E Cinotti1,2, D Metze3, M Battistella4, P G Calzavara-Pinton5, K Damevska6, G Girolomoni7, J André8, J-L Perrot2, W Kempf9, B Cavelier-Balloy10. 1. IRCSS-AOU S. Martino-IST, Department of Health Sciences, DISSAL, Section of Dermatology, University of Genoa, Genoa, Italy. 2. Section of Dermatology, University Hospital of Saint-Etienne, Saint-Etienne, France. 3. Department of Dermatology, University Hospital, Munster, Germany. 4. Department of Pathology, Hôpital Saint Louis, Assistance Publique-Hôpitaux de Paris, Paris, France. 5. Department of Dermatology, University Hospital Spedali Civili, Brescia, Italy. 6. Clinic of Dermatology, Medical Faculty University "Ss Cyril and Methodius", Skopje, Republic of Macedonia. 7. Department of Medicine, Section of Dermatology and Venereology, University of Verona, Verona, Italy. 8. Department of Dermatology, CHU-Saint Pierre, Free University of Brussels, Brussels, Belgium. 9. Kempf and Pfaltz, Histological Diagnostics, and Dept. of Dermatology, University Hospital Zürich, Zürich, Switzerland. 10. Service de Dermatopathologie, Paris, France.
Abstract
BACKGROUND: The prognostic and therapeutic features of scleredema are poorly documented. OBJECTIVES: 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.
BACKGROUND: The prognostic and therapeutic features of scleredema are poorly documented. OBJECTIVES: 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: Aikaterini Kyriakou; Sofia-Chrysovalantou Zagalioti; Elizabeth Lazaridou; Aikaterini Patsatsi Journal: J Family Med Prim Care Date: 2021-02-27