Scleroderma
From Wikipedia, the free encyclopedia
| Scleroderma | |
| Classification and external resources | |
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| ICD-10 | L94.0-L94.1, M34. |
| ICD-9 | 701.0 710.1 |
Scleroderma is a chronic autoimmune disease characterized by a hardening[1] or sclerosis[2] in the skin or other organs. The localized type of the disease, known as "morphea",[3] while disabling, tends not to be fatal. The systemic type or systemic sclerosis, the generalized type of the disease, can be fatal, as a result of heart, kidney, lung or intestinal damage.[4] It is currently not fully understood what exactly causes this disease, although there are various theories.
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[edit] Types
Scleroderma is characterized by the appearance of circumscribed or diffuse, hard, smooth, ivory-colored areas that are immobile, and which give the appearance of hidebound skin, a disease occurring in both localized and systemic forms:[5]
[edit] Incidence
This disease is found among all races worldwide, but women are four times more likely to contract scleroderma than men. In the United States, approximately one person in 1,000 is affected. Children rarely suffer the systemic type, but localized scleroderma is common. Most adults are diagnosed after their 30th birthday and before age 50. The disease has high rates among the native American Choctaw tribe and African-American females.[6]
[edit] Diagnosis
Typical scleroderma is classically defined as symmetrical skin thickening, with about 90% of cases also presenting with Raynaud's phenomenon, nail-fold capillary changes, and anti-nuclear antibodies. Patients may or may not experience systemic organ involvement. Atypical scleroderma may show any variation of these changes without skin changes or with finger swelling only.[7][8] Additional symptoms of scleroderma typically present themselves within two years of Raynaud's phenomenon.[6]
Laboratory testing can show anti-topoisomerase antibodies (causing a diffuse systemic form), or anti-centromere antibodies (causing a limited systemic form, and the CREST syndrome). Other autoantibodies can be seen, such as anti-U3 or anti-RNA polymerase.
Severe complications from Scleroderma include:
- Heart - Untreated high blood pressure strains the heart, irregular heart rhythm and enlargement of the heart lead to heart failure.
- Kidney - Blood vessel damage causes blood pressure to rise, which untreated, will result in brain swelling, headaches, retinal damage and seizures.
- Lung - Two-thirds of all patients suffer from respiratory problems such as shortness of breath, coughing, difficulty breathing, alveolitis (inflammation of lung air sacs), pneumonia and cancer.
- Digestive - Esophagus damage can make it difficult to swallow food, and acid reflux is common. A sluggish intestine may cause pain & bloating; undigested food can result in diarrhea, weight loss and anemia.
- Skin & Joints - Carpal tunnel syndrome is common, as is muscle weakness, joint pain & stiffness.[6]
[edit] Treatment
There is no direct cure for scleroderma. Because the exact cause is unknown, any treatment is patient-specific and aimed at ameliorating symptoms of the disease. For example, patients who experience Raynaud's Phenomenon may be treated with agents to increase blood flow to the fingers, including nifedipine, amlodipine, diltiazem, felodipine, or nicardipine.
Fibrosis of the skin has been treated with varying degrees of success with agents such as d-penicllamine, colchicine, PUVA, Relaxin, and cyclosporine.
Because scleroderma is an autoimmune disease, one of the major pillars of treatment involves the use of immunosuppressive agents. These drugs include methotrexate, cyclophosphamide, azathioprine, and mycophenolate.[9][10]
Patients with lung involvement benefit from Oxygen therapy, which increases oxygen saturation in tissues damaged by the progression of scleroderma. The additional oxygen in the blood reduces the effort of the heart, decreasing shortness of breath.[11]
[edit] Prognosis
Individuals with morphea or limited scleroderma have a relatively positive outlook. They will usually succumb to another disease, not the scleroderma. Those with very widespread skin and organ involvement (systemic) have a negative prognosis. More women have scleroderma, but the disease kills more men. Following diagnosis, two-thirds of patients live at least 11 years. The higher the patient's age at diagnosis, the more likely they are to die from the disease.
[edit] History
Cases of skin disease similar to scleroderma may be found in the writings of Hippocrates as far back as 460–370 B.C. Oribasius (325–403 A.D.) and Paulus Agineta (625–690 A.D.), also wrote on the subject. It is difficult for us to know if these were truly examples of scleroderma because the descriptions were inexact.
The first definite description of the disease was by Carlo Curzio in a monograph published in Naples in 1753. This account produced considerable interest in French and English medical circles.
The account concerns a young woman of 17 named Patrizia Galiera, who was admitted to the hospital and assigned to Dr. Curzio. Her symptoms as described by the doctor involved hardness of the skin (differing in degree from place to place), tightness around the mouth, and hardness around the neck. He noted loss of warmth in the skin but no other problem in pulse, respiration, or digestion.
Much of the report contains details of the treatment, which included warm milk and vapor baths, bleeding from the foot, and small doses of quicksilver. After 11 months, the skin became soft and flexible, and all natural functions were restored.
Curzio's observations were published in French in 1755 and aroused considerable interest. The early dermatological texts of R. William in London (1808) and his student, J. L. Alibert, in Paris (1818) referred to Curzio's observation.[12]
[edit] Additional images
 Left arm of Scleroderma patient, showing skin lesions |
[edit] References
- ^ Scleroderma at Dorland's Medical Dictionary
- ^ MeSH Systemic+Scleroderma
- ^ MeSH Scleroderma,+Localized
- ^ Klippel, John H.. Primer On the Rheumatic Diseases 11ED. Atlanta, GA: Arthritis Foundation. ISBN 1-912423-16-2.
- ^ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. Page 169-172. ISBN 0721629210.
- ^ a b c [1] The Free Dictionary, Medical terms, Scleroderma
- ^ Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum. 1980; 23:581-90.
- ^ Barnett A, Miller M, Littlejohn G. The Diagnosis and Classification of Scleroderma. Postgraduate Medical Journal. 1988; 64:121-125.
- ^ Fleischmajer,R., Sapadin, AN.Treatment of Scleroderma. Arch Dermatol. 2002; 138:99 - 104
- ^ Leighton, C. Drug Treatment of Scleroderma. Drugs. 2001; 61(3): 419-27.
- ^ [2] International Scleroderma Network, Lung, Oxygen therapy
- ^ A Brief History of Scleroderma, http://www.scleroderma.org/medical/other_articles/Coyle_2001_4.shtm
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