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Vedat Inal - One of the best experts on this subject based on the ideXlab platform.
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Therapy resistant idiopathic Scleredema: an underlying pathology not always present.
European journal of rheumatology, 2015Co-Authors: Melike Kalfa, Hayriye Koçanaoğulları, Figen Yargucu Zihni, Gonca Karabulut, Hakan Emmungil, Vedat InalAbstract:Scleredema is a rare connective tissue disorder of unknown pathogenesis. Three types of Scleredema have been described, based on its association with postinfection, monoclonal gammopathy and diabetes mellitus. We report herein a case of Scleredema which the diagnosis didn't get specified. The patient was followed regularly for 13 years and did not respond to various combinations of immunosuppressants and psoralen plus ultraviolet A therapy. Treatment of Scleredema is quite difficult and of limited success. At present, there is no proved treatment for this disease.
Melike Kalfa - One of the best experts on this subject based on the ideXlab platform.
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Therapy resistant idiopathic Scleredema: an underlying pathology not always present.
European journal of rheumatology, 2015Co-Authors: Melike Kalfa, Hayriye Koçanaoğulları, Figen Yargucu Zihni, Gonca Karabulut, Hakan Emmungil, Vedat InalAbstract:Scleredema is a rare connective tissue disorder of unknown pathogenesis. Three types of Scleredema have been described, based on its association with postinfection, monoclonal gammopathy and diabetes mellitus. We report herein a case of Scleredema which the diagnosis didn't get specified. The patient was followed regularly for 13 years and did not respond to various combinations of immunosuppressants and psoralen plus ultraviolet A therapy. Treatment of Scleredema is quite difficult and of limited success. At present, there is no proved treatment for this disease.
Jeffrey J. Newmark - One of the best experts on this subject based on the ideXlab platform.
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Severe Scleredema diabeticorum and insulin resistance.
The Journal of the American Board of Family Practice, 1995Co-Authors: Bruce E. Wilson, Jeffrey J. NewmarkAbstract:Scleredema, an unusual disorder of the skin, is associated with symmetric induration and thickening of the skin generally on the posterior neck and the upper back.1-4 Scleredema is classified. into two types, Scleredema adultorum of Buschke and Scleredema diabeticorum.4,5 Scleredema adultorum of Buschke is more commonly diagnosed in children, despite the term adultorum, and is 'usually preceded by an acute infection, often streptococcal. Clinically, this type can easily be recognized by observing the patient's indurated, nonpitting skin. Although there have been cases in which internal organs are involved by Scleredema, the disease usually enters remission without any treatment within 18 months. Scleredema diabeticorum is generally associated with non-insulin-dependent diabetes mellitus (NIDDM).1,6,7 While this second type of Scleredema involves the upper back and neck in a manner similar to the Buschke type, Scleredema diabeticorum can involve a more extensive region of the body, extremities, and internal organs. Scleredema diabeticorum can persist for many years, and there is no effective therapy. Histologically, the dermis is characterized by hyperplasia of collagen and increased accumulation of gIycosaminoglycans and hyaluronic acid in both types of Scleredema.
Terry L. Moore - One of the best experts on this subject based on the ideXlab platform.
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Scleredema Adultorum of Buschke: A Case Report and Review of the Literature
Seminars in arthritis and rheumatism, 2006Co-Authors: William H. Beers, Ince A, Terry L. MooreAbstract:Objectives To present a case of Scleredema adultorum of Buschke associated with hypergammaglobulinemia and review the literature pertaining to this disease. Methods Search of MEDLINE (PubMed) was performed using the words "Scleredema Adultorum." Only cases in the English language, with pertinent clinical information for analysis, were included. Results We present a case of Scleredema associated with hypergammaglobulinemia. The skin findings had been progressive for 2 years before he was diagnosed with Scleredema. Our patient was found to have a B-cell lymphoma before being diagnosed with Scleredema. The progression of skin thickening halted with no apparent correlation to immunosuppressive therapies or chemotherapeutic agents. A total of 165 cases of Scleredema adultorum are described in the literature. There are 3 types of Scleredema adultorum. Type 1 is usually preceded by a febrile episode and resolves spontaneously. Type 2 is associated with developing paraproteinemias including multiple myeloma. Type 3 is associated with diabetes mellitus. Conclusions Scleredema adultorum of Buschke is a rare disorder which is sometimes associated with hypergammaglobulinemia; our patient had a B-cell lymphoma. Physicians should suspect Scleredema in any patient with diffuse skin thickening where the hands and feet are spared, particularly if diabetes or a preceding febrile episode were present. Once the diagnosis of Scleredema is made, evidence for the presence of hypergammaglobulinemia should be sought.
Bernard Vialettes - One of the best experts on this subject based on the ideXlab platform.
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Scleredema adultorum of Buschke: an under recognized skin complication of diabetes
Diabetes & Metabolism, 2006Co-Authors: C. Meguerditchian, P. Jacquet, Sophie Béliard, T Benderitter, René Valéro, F Carsuzza, Bernard VialettesAbstract:Summary Scleredema of Buschke or Scleredema diabetorum is a skin complication of diabetes with deposits of collagen and aminoglycans in the dermis. This disease characterized by thickening and hardening of the skin, is usually localized in nape, back and shoulder areas. Consequences could be a decrease in motility of the shoulders and an impairment of respiratory function. Other possible complications are sleep apnoea syndrome and monoclonal gammapathy. Type 1 or type 2 diabetes may be associated with Scleredema of Buschke in more than 50% of cases. Diabetes-related risk factors are long duration of the disease, presence of microangiopathy, overweight and need of insulin. Various specific treatments proposed in the literature are poorly validated. In most severe cases, radiation therapy may be useful.