Morphea

Morphea
Classification and external resources
Specialty	dermatology
ICD-10	L94.0
ICD-9-CM	701.0
DiseasesDB	8351
eMedicine	derm/272
Patient UK	Morphea
MeSH	D012594

Morphea, also known as "localized scleroderma", or "circumscribed scleroderma", involves isolated patches of hardened skin with no internal organ involvement.^[1]:130

Classification

• Morphea–lichen sclerosus et atrophicus overlap is characterized by both lesions of morphea and lichen sclerosus et atrophicus, most commonly seen in women.^[2]:171
• Generalized morphea is characterized by widespread indurated plaques and pigmentary changes, sometimes associated with muscle atrophy, but without visceral involvement.^[2]:171
• Morphea profunda involves deep subcutaneous tissue, including fascia, and there is a clinical overlap with eosinophilic fasciitis, eosinophilia-myalgia syndrome, and the Spanish toxic oil syndrome.^[2]:171 Morphea profunda shows little response to corticosteroids and tends to run a more chronic debilitating course.^[2]:171
• Pansclerotic morphea is manifested by sclerosis of the dermis, panniculus, fascia, muscle, and at times, the bone, all causing disabling limitation of motion of joints.^[2]:171
• Linear scleroderma is a type of localised scleroderma^[3] which is an autoimmune disease characterized by a line of thickened skin which can affect the bones and muscles underneath it. It most often occurs in the arms, legs, or forehead, and may occur in more than one area. It is also most likely to be on just one side of the body. Linear scleroderma generally first appears in young children.^[2]
• Frontal linear scleroderma (also known as en coup de sabre or morphea en coup de sabre) is a type of linear scleroderma characterized by a linear band of atrophy and a furrow in the skin that occurs in the frontal or frontoparietal scalp.^[4][5] Multiple lesions of en coup de sabre may coexist in a single patient, with one report suggesting that the lesions followed Blaschko's lines.^[5] It gets its name from the perceived similarity to a sabre wound.^[6]

Frontal linear scleroderma

• Atrophoderma of Pasini and Pierini (also known as "Dyschromic and atrophic variation of scleroderma,"^[4] "Morphea plana atrophica,"^[4] "Sclérodermie atrophique d'emblée"^[4]) is a disease characterized by large lesions with a sharp peripheral border dropping into a depression with no outpouching, which, on biopsy, elastin is normal, while collagen may be thickened.^[7] Atrophoderma of Pasini and Pierini affects less than 200,000 Americans and is classified as a rare disease by http://rarediseases.info.nih.gov...") The disease results in round or oval patches of hyper-pigmented skin. The darkened skin patches may sometimes have a bluish or purplish hue when they first appear and are often smooth to the touch and hairless.

Signs and symptoms

Frontal linear scleroderma

Morphea is most often presents as macules or plaques a few centimeters in diameter, but also may occur as bands or in guttate lesions or nodules.^[2]:171

Morphea 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. .^[8] 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.^[9]

Cause

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.^[9] Tests for autoantibodies associated with morphea have shown results in higher frequencies of anti-histone and anti-topoisomerase IIa antibodies.^[10] 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.^[11][12][13]

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. Some have tried prescription vitamian-D with success. 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.

Epidemiology

Morphea is a form of scleroderma that is more common in women than men, in a ratio 3:1.^[14] Morphea occurs in childhood as well as in adult life.^[2]

Morphea is an uncommon condition that is thought to affect 2 to 4 in 100,000 people.^[15] 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.

See also

• List of cutaneous conditions
• Frontal linear scleroderma (morphea en coup de sabre)

References

1. Fitzpatrick, Thomas B. (2005). Fitzpatrick's color atlas and synopsis of clinical dermatology (5th ed.). New York: McGraw-Hill Medical Pub. Division. ISBN 0-07-144019-4.
2. James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. Page 171. ISBN 0-7216-2921-0.
3. "linear scleroderma" at Dorland's Medical Dictionary
4. Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
5. Katz, KA (October 2003). "Frontal linear scleroderma (en coup de sabre).". Dermatology online journal 9 (4): 10. PMID 14594583.
6. "coup de sabre" at Dorland's Medical Dictionary
7. Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). Page 1029. McGraw-Hill. ISBN 0-07-138076-0.
8. Peterson LS, Nelson AM, Su WP; Nelson; Su (1995). "Classification of morphea (localized scleroderma)". Mayo Clin. Proc. 70 (11): 1068–76. doi:10.4065/70.11.1068. PMID 7475336.
9. Zulian F, Athreya BH, Laxer R (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.
10. Hayakawa I, Hasegawa M, Takehara K, Sato S; Hasegawa; Takehara; Sato (2004). "Anti-DNA topoisomerase IIalpha autoantibodies in localized scleroderma". Arthritis Rheum. 50 (1): 227–32. doi:10.1002/art.11432. PMID 14730620.
11. Majeed M, Al-Mayouf SM, Al-Sabban E, Bahabri S; Al-Mayouf; Al-Sabban; Bahabri (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.
12. Bonifati C, Impara G, Morrone A, Pietrangeli A, Carducci M; Impara; Morrone; Pietrangeli; Carducci (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.
13. González-López MA, Drake M, González-Vela MC, Armesto S, Llaca HF, Val-Bernal JF; Drake; González-Vela; Armesto; Llaca; Val-Bernal (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.
14. Laxer RM, Zulian F; Zulian (2006). "Localized scleroderma". Curr Opin Rheumatol 18 (6): 606–13. doi:10.1097/01.bor.0000245727.40630.c3. PMID 17053506.
15. Peterson LS, Nelson AM, Su WP, Mason T, O'Fallon WM, Gabriel SE; Nelson; Su; Mason; O'Fallon; Gabriel (1997). "The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993". J. Rheumatol. 24 (1): 73–80. PMID 9002014.

External links

• UT-Southwestern Medical Center at Dallas Morphea Registry
• JAMA Dermatology Patient Page. Morphea (Localized Scleroderma. Nicole M. Fett, MD. JAMA Dermatol. 2013;149(9):1124. doi:10.1001/jamadermatol.2013.5079. September 2013