Scleredema. A multicentre study of characteristics, comorbidities, course and therapy in 44 patients
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 m...
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Published in | Journal of the European Academy of Dermatology and Venereology Vol. 29; no. 12; pp. 2399 - 2404 |
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Main Authors | , , , , , , , , , , , |
Format | Journal Article |
Language | English |
Published |
England
Blackwell Publishing Ltd
01.12.2015
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Subjects | |
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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. |
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AbstractList | The prognostic and therapeutic features of scleredema are poorly documented.
To describe the characteristics of patients with scleredema regarding demographics, clinical characteristics, comorbidities, therapeutic interventions and course.
We conducted a retrospective multicentre study.
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.
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. BACKGROUNDThe prognostic and therapeutic features of scleredema are poorly documented.OBJECTIVESTo describe the characteristics of patients with scleredema regarding demographics, clinical characteristics, comorbidities, therapeutic interventions and course.METHODSWe conducted a retrospective multicentre study.RESULTSWe 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.CONCLUSIONSScleredema 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. 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 ( UVA 1 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. |
Author | Perrot, J.-L. Rongioletti, F. Metze, D. André, J. Kaiser, F. Battistella, M. Girolomoni, G. Cinotti, E. Damevska, K. Cavelier-Balloy, B. Calzavara-Pinton, P.G. Kempf, W. |
Author_xml | – sequence: 1 givenname: F. surname: Rongioletti fullname: Rongioletti, F. email: franco.rongioletti@unige.it organization: IRCSS-AOU S. Martino-IST, Department of Health Sciences, DISSAL, Section of Dermatology, University of Genoa, Genoa, Italy – sequence: 2 givenname: F. surname: Kaiser fullname: Kaiser, F. organization: IRCSS-AOU S. Martino-IST, Department of Health Sciences, DISSAL, Section of Dermatology, University of Genoa, Genoa, Italy – sequence: 3 givenname: E. surname: Cinotti fullname: Cinotti, E. organization: IRCSS-AOU S. Martino-IST, Department of Health Sciences, DISSAL, Section of Dermatology, University of Genoa, Genoa, Italy – sequence: 4 givenname: D. surname: Metze fullname: Metze, D. organization: Department of Dermatology, University Hospital, Munster, Germany – sequence: 5 givenname: M. surname: Battistella fullname: Battistella, M. organization: Department of Pathology, Hôpital Saint Louis, Assistance Publique-Hôpitaux de Paris, Paris, France – sequence: 6 givenname: P.G. surname: Calzavara-Pinton fullname: Calzavara-Pinton, P.G. organization: Department of Dermatology, University Hospital Spedali Civili, Brescia, Italy – sequence: 7 givenname: K. surname: Damevska fullname: Damevska, K. organization: Clinic of Dermatology, Medical Faculty University "Ss Cyril and Methodius", Skopje, Republic of Macedonia – sequence: 8 givenname: G. surname: Girolomoni fullname: Girolomoni, G. organization: Department of Medicine, Section of Dermatology and Venereology, University of Verona, Verona, Italy – sequence: 9 givenname: J. surname: André fullname: André, J. organization: Department of Dermatology, CHU-Saint Pierre, Free University of Brussels, Brussels, Belgium – sequence: 10 givenname: J.-L. surname: Perrot fullname: Perrot, J.-L. organization: Section of Dermatology, University Hospital of Saint-Etienne, Saint-Etienne, France – sequence: 11 givenname: W. surname: Kempf fullname: Kempf, W. organization: Kempf and Pfaltz, Histological Diagnostics, and Dept. of Dermatology, University Hospital Zürich, Zürich, Switzerland – sequence: 12 givenname: B. surname: Cavelier-Balloy fullname: Cavelier-Balloy, B. organization: Service de Dermatopathologie, Paris, France |
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Notes | ArticleID:JDV13272 ark:/67375/WNG-B82G0J64-4 istex:24D9B940BED244E50C2E392A7D8533C4CB01F24B Conflicts of interest Funding sources None declared. ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23 |
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Snippet | Background
The prognostic and therapeutic features of scleredema are poorly documented.
Objectives
To describe the characteristics of patients with scleredema... The prognostic and therapeutic features of scleredema are poorly documented. To describe the characteristics of patients with scleredema regarding... Abstract Background The prognostic and therapeutic features of scleredema are poorly documented. Objectives To describe the characteristics of patients with... BACKGROUNDThe prognostic and therapeutic features of scleredema are poorly documented.OBJECTIVESTo describe the characteristics of patients with scleredema... |
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SubjectTerms | Adrenal Cortex Hormones - therapeutic use Adult Aged Comorbidity Diabetes Mellitus, Type 2 - epidemiology Dyslipidemias - epidemiology Female Humans Immunosuppressive Agents - therapeutic use Male Middle Aged Obesity - epidemiology Paraproteinemias - epidemiology PUVA Therapy Retrospective Studies Scleredema Adultorum - drug therapy Scleredema Adultorum - epidemiology Survival Rate Young Adult |
Title | Scleredema. A multicentre study of characteristics, comorbidities, course and therapy in 44 patients |
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