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Scleredema adultorum of Buschke: a case report and review of the literature.
MedLine Citation:
PMID:  16765712     Owner:  NLM     Status:  MEDLINE    
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.
William H Beers; Akgun Ince; Terry L Moore
Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Seminars in arthritis and rheumatism     Volume:  35     ISSN:  0049-0172     ISO Abbreviation:  Semin. Arthritis Rheum.     Publication Date:  2006 Jun 
Date Detail:
Created Date:  2006-06-12     Completed Date:  2006-11-07     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  1306053     Medline TA:  Semin Arthritis Rheum     Country:  United States    
Other Details:
Languages:  eng     Pagination:  355-9     Citation Subset:  IM    
Division of Rheumatology, Department of Internal Medicine, Saint Louis University School of Medicine, St. Louis, MO 63104, USA.
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MeSH Terms
Biopsy / methods
Blood Protein Electrophoresis
Bone Marrow Examination
Hypergammaglobulinemia / etiology
Lymphoma, B-Cell / complications*,  diagnosis,  drug therapy
Scleredema Adultorum / complications*,  diagnosis,  drug therapy
Skin / pathology*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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