Morphea

Morphea is a medical term for localized scleroderma. The disease involves isolated patches of hardened skin - there generally is no internal organ involvement.^[1]

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Background and classification

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 discriminates from systemic sclerosis by its supposed lack of internal organ involvement. The most widely used classification divides morphea into five general subtypes: plaque morphea, generalized morphea, linear scleroderma, bullous morphea, and deep morphea (1). 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 (2).

Epidemiology

Morphea is an uncommon condition that is thought to affect 1 in 100,000 people(3). 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 (4).

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 (2). Tests for autoantibodies associated with morphea have shown results in higher frequencies of anti-histone and anti-topoisomerase IIa antibodies (5). 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 (6,7,8).

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

• 1) Peterson LS, Nelson AM, Su WPD. Subspecialty clinics: rheumatology and dermatology classifcation of morphea (localized scleroderma). Mayo Clin Proc 1995; 70:1068-1076.

• 2) Zulian F, Athreya BH, Laxer et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children: an international study. Rheumatology (oxford) 2006: 45:614-620.

• 3) Peterson LS, Nelson AM, Su WPD, et al. The epidemiology of morphea (localized scleroderma) in Olmsted County. J Rheumatology 1997; 24:73-80.

• 4) Laxer RM and Zulian F. Localized scleroderma. Review. Curr opin Rheum 2006 18: 606-613.

• 5) 28. Hayakawa, Hasegawa, Takehara, Sato. Anti-DNA topoIIA autoabs in localized scleroderma. Arthritis Rheum 2004 Jan 50(1): 227-32.

• 6) Majeed M, Al Mayouf SM, Al-Sabban E, Bahabri S. Coexistent linear scleroderma and juvenile SLE. Ped Derm 2000 Nov-Dec; 17 (6): 456-9.

• 7) Bonifati C, Carducci M. Simultaneous occurrence of linear scleroderma and homolateral segmental vitiligo. J Eur Acad Derm Ven 2006 Jan; 20(1): 63-5

• 8) Marcos A Gonzalez-Lopez, Marta Drake J. Fernando val-bernal. Generalized morphea and primar biliary cirrhosis coexisting in a male patient. J of Derm 2006; 33:709-713.