Morphea: Difference between revisions
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==Overview== | |||
Morphea, also known as localized scleroderma, is a thickening and hardening of the skin and subcutaneous tissues from excessive collagen deposition. Morphea includes specific conditions ranging from very small plaques only involving the skin to widespread disease causing functional and cosmetic deformities. Morphea involves isolated patches of hardened skin and discriminates from systemic sclerosis by its supposed lack of internal organ involvement.<ref>[http://premium.edition.cnn.com/HEALTH/library/DS/00718.html Morpea] CNN.com, (May 05, 2006).</ref> | '''Morphea''', also known as localized scleroderma, is a thickening and hardening of the skin and subcutaneous tissues from excessive collagen deposition. Morphea includes specific conditions ranging from very small plaques only involving the skin to widespread disease causing functional and cosmetic deformities. Morphea involves isolated patches of hardened skin and discriminates from systemic sclerosis by its supposed lack of internal organ involvement.<ref>[http://premium.edition.cnn.com/HEALTH/library/DS/00718.html Morpea] CNN.com, (May 05, 2006).</ref> | ||
==Classification== | ==Classification== | ||
Revision as of 15:32, 7 April 2009
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| ICD-10 | L94.0 |
| ICD-9 | 701.0 |
| DiseasesDB | 8351 |
| eMedicine | derm/272 |
| MeSH | D012594 |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Morphea, also known as localized scleroderma, is a thickening and hardening of the skin and subcutaneous tissues from excessive collagen deposition. Morphea includes specific conditions ranging from very small plaques only involving the skin to widespread disease causing functional and cosmetic deformities. Morphea involves isolated patches of hardened skin and discriminates from systemic sclerosis by its supposed lack of internal organ involvement.[1]
Classification
The most widely used classification divides morphea into five general subtypes: plaque morphea, generalized morphea, linear scleroderma, bullous morphea, and deep morphea.[2] This classification scheme does not include the mixed form of morphea in which different morphologies of skin lesions are present in the same individual. Up to 15% of morphea patients may fall into this previously unrecognized category.[3]
Epidemiology
Morphea is an uncommon condition that is thought to affect 1 in 100,000 people.[4] Adequate studies on the incidence and prevalence have not been performed. Morphea also may be under-reported as physicians may be unaware of this disorder and smaller morphea plaques may be less often referred to a dermatologist or rheumatologist. As in many other connective tissue or autoimmune disorders, morphea mainly involves women with a W:M ratio of 3:1.[5]
Etiology
Physicians and scientists do not know what causes morphea. Case reports and observational studies suggest there is a higher frequency of family history of autoimmune diseases in patients with morphea.[3] Tests for autoantibodies associated with morphea have shown results in higher frequencies of anti-histone and anti-topoisomerase IIa antibodies.[6] Case reports of morphea co-existing with other systemic autoimmune diseases such as primary biliary cirrhosis, vitiligo, and systemic lupus erythematosus lend support to morphea as an autoimmune disease.[7][8][9]
Treatment
Throughout the years, many different treatments have been tried for morphea including topical, intra-lesional, and systemic corticosteroids. Antimalarials such as hydroxychloroquine or chloroquine have been used. Other immunomodulators such as methotrexate, topical tacrolimus, and penicillamine have been tried. Ultraviolet A (UVA) light, with or without psoralens have also been tried. UVA-1, a more specific wavelength of UVA light, is able to penetrate the deeper portions of the skin and thus, thought to soften the plaques in morphea by acting in two fashions:
- 1) by causing a systemic immunosuppression from UV light.
- 2) by inducing enzymes that naturally degrade the collagen matrix in the skin as part of natural sun-aging of the skin.
As with all of these treatments for morphea, the difficulty in assessing outcomes in an objective way has limited the interpretation of most studies involving these treatment modalities.
References
- ↑ Morpea CNN.com, (May 05, 2006).
- ↑ Peterson LS, Nelson AM, Su WP (1995). "Classification of morphea (localized scleroderma)". Mayo Clin. Proc. 70 (11): 1068–76. PMID 7475336.
- ↑ 3.0 3.1 Zulian F, Athreya BH, Laxer R; et al. (2006). "Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study". Rheumatology (Oxford). 45 (5): 614–20. doi:10.1093/rheumatology/kei251. PMID 16368732.
- ↑ Peterson LS, Nelson AM, Su WP, Mason T, O'Fallon WM, Gabriel SE (1997). "The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993". J. Rheumatol. 24 (1): 73–80. PMID 9002014.
- ↑ Laxer RM, Zulian F (2006). "Localized scleroderma". Curr Opin Rheumatol. 18 (6): 606–13. doi:10.1097/01.bor.0000245727.40630.c3. PMID 17053506.
- ↑ Hayakawa I, Hasegawa M, Takehara K, Sato S (2004). "Anti-DNA topoisomerase IIalpha autoantibodies in localized scleroderma". Arthritis Rheum. 50 (1): 227–32. doi:10.1002/art.11432. PMID 14730620.
- ↑ Majeed M, Al-Mayouf SM, Al-Sabban E, Bahabri S (2000). "Coexistent linear scleroderma and juvenile systemic lupus erythematosus". Pediatr Dermatol. 17 (6): 456–9. doi:10.1046/j.1525-1470.2000.01820.x. PMID 11123778.
- ↑ Bonifati C, Impara G, Morrone A, Pietrangeli A, Carducci M (2006). "Simultaneous occurrence of linear scleroderma and homolateral segmental vitiligo". J Eur Acad Dermatol Venereol. 20 (1): 63–5. doi:10.1111/j.1468-3083.2005.01336.x. PMID 16405610.
- ↑ González-López MA, Drake M, González-Vela MC, Armesto S, Llaca HF, Val-Bernal JF (2006). "Generalized morphea and primary biliary cirrhosis coexisting in a male patient". J. Dermatol. 33 (10): 709–13. doi:10.1111/j.1346-8138.2006.00165.x. PMID 17040502.