Scleromyxedema : A chronic progressive systemic disease.
Summary of "Scleromyxedema : A chronic progressive systemic disease."
BACKGROUND:
Scleromyxedema is a rare connective tissue disease that may affect numerous internal organs in addition to the skin. The disease is almost exclusively associated with monoclonal gammopathy. MATERIAL AND
METHODS:
This retrospective study summarizes the clinical characteristics of four patients with scleromyxedema. In all of the patients a systematic serological and apparative check-up was performed.
RESULTS:
The mean age of the four patients (three women and one man) was 51 years. In all cases, monoclonal gammopathy (3 cases of IgG lambda and 1 case of IgG kappa) was involved. In one patient, skin lesions were restricted to the upper part of the body and three patients had generalized disease. The internal organs of all patients were affected with fibrosis of the lungs, myositis and arthritis, peripheral polyneuropathy and hypomotility of the esophagus. The most effective forms of treatment in this patient collective were dexamethasone-pulse therapy, intravenous immunoglobulins and bortezomib. All patients had recurrences after finishing therapy. The mean observation period after the initial diagnosis of scleromyxedena was 6.25 years (range 2-11 years).
CONCLUSION:
Scleromyxedema is a rare multisystemic disease. The heterogeneous affection of internal organs necessitates a comprehensive check-up. The response to recently published treatment strategies is low and recurrences after finishing therapy are frequent.
Affiliation
Klinik für Dermatologie, Venerologie und Allergologie, Ruhr-Universität Bochum im St. Josef Hospital, Gudrunstr. 56, 44791, Bochum, Deutschland, A.Kreuter@derma.de.
Journal Details
This article was published in the following journal.
Name: Zeitschrift fur Rheumatologie
ISSN: 1435-1250
Pages:
Links
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/22806700
- DOI: http://dx.doi.org/10.1007/s00393-012-0997-5
Medical and Biotech [MESH] Definitions
Scleromyxedema
A connective tissue disorder characterized by widespread thickening of SKIN with a cobblestone-like appearance. It is caused by proliferation of FIBROBLASTS and deposition of MUCIN in the DERMIS in the absence of thyroid disease. Most scleromyxedema cases are associated with a MONOCLONAL GAMMOPATHY, immunoglobulin IgG-lambda.
Multiple Sclerosis, Chronic Progressive
A form of multiple sclerosis characterized by a progressive deterioration in neurologic function which is in contrast to the more typical relapsing remitting form. If the clinical course is free of distinct remissions, it is referred to as primary progressive multiple sclerosis. When the progressive decline is punctuated by acute exacerbations, it is referred to as progressive relapsing multiple sclerosis. The term secondary progressive multiple sclerosis is used when relapsing remitting multiple sclerosis evolves into the chronic progressive form. (From Ann Neurol 1994;36 Suppl:S73-S79; Adams et al., Principles of Neurology, 6th ed, pp903-914)
Pancreatitis, Alcoholic
Acute or chronic INFLAMMATION of the PANCREAS due to excessive ALCOHOL DRINKING. Alcoholic pancreatitis usually presents as an acute episode but it is a chronic progressive disease in alcoholics.
Bronchitis, Chronic
A subcategory of CHRONIC OBSTRUCTIVE PULMONARY DISEASE. The disease is characterized by hypersecretion of mucus accompanied by a chronic (more than 3 months in 2 consecutive years) productive cough. Infectious agents are a major cause of chronic bronchitis.
Coccidioidomycosis
Infection with a fungus of the genus COCCIDIOIDES, endemic to the SOUTHWESTERN UNITED STATES. It is sometimes called valley fever but should not be confused with RIFT VALLEY FEVER. Infection is caused by inhalation of airborne, fungal particles known as arthroconidia, a form of FUNGAL SPORES. A primary form is an acute, benign, self-limited respiratory infection. A secondary form is a virulent, severe, chronic, progressive granulomatous disease with systemic involvement. It can be detected by use of COCCIDIOIDIN.
PubMed Articles
Scleroderma is a rare systemic autoimmune disease with multiple organ manifestations, including skin fibrosis. The groups of disorders classified as scleroderma mimics share the common thread of skin...
A case of chronic progressive myelopathy.
Acute myelitis and optic neuritis are the main clinical features of patients with neuromyelitis optica (NMO), which usually appears as a relapsing-remitting course of disease that worsens over days an...
Progressive multiple sclerosis is not associated with chronic cerebrospinal venous insufficiency.
Chronic cerebrospinal venous insufficiency (CCSVI) had been suggested to play a major pathogenetic role in multiple sclerosis (MS), but recent data on early stages of MS have not confirmed this theory...
The globe: infection, inflammation, and systemic disease.
Infection, inflammation, and systemic diseases affecting the globe encompass a broad range of pathologies which may ultimately lead to progressive vision loss. Clinical symptomatology varies from the...
Abstract Long-term therapy with systemic corticosteroids is not recommended in the treatment of chronic obstructive pulmonary disease (COPD). However, experience demonstrates that some patients rece...
Clinical Trials
Thalidomide for Decreasing Collagen Biosynthesis in People With Progressive Systemic Sclerosis
Progressive systemic sclerosis (SSc) is an immune-based disease that causes abnormal connective tissue growth of the skin and internal organs. At this point, there are no effective therapi...
The study tests the hypothesis that systemic and renal nitric oxide availability is changed in polycystic kidney disease and chronic glomerulonephritis.
Chronic obstructive pulmonary disease is associated with a low grade systemic inflammatory process. Systemic inflammation is hypothesized to maintain cardiovascular morbidity and mortality...
Intradialytic Progressive Resistance Training for Maintenance Haemodialysis Patients
The purpose of this study is to investigate the effects of a 12 week progressive resistance training during haemodialysis on muscle quantity and physical functioning in chronic kidney dise...
Evaluation of a Patient Education Program for Ankylosing Spondylitis
Long term disease management is essential for Ankylosing Spondylitis (AS), a chronic progressive systemic inflammatory disease of the axial skeleton. Extensive information about the diseas...
