Morphea

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Jesus Rosario Hernandez, M.D. [2],Kiran Singh, M.D. [3].

Synonyms and keywords: Localized scleroderma.

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.

Classification

The most widely used classification divides morphea into five general subtypes: plaque morphea, generalized morphea, linear scleroderma, bullous morphea, and deep morphea. 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.

Epidemiology

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

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. Tests for autoantibodies associated with morphea have shown results in higher frequencies of anti-histone and anti-topoisomerase IIa antibodies. 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.

Diagnoses of Morphea should be Distinguished from

• Annular lichenoid dermatitis of youth
• Ataxia-telangiectasia
• Atrophoderma of pasini and pierini
• Carcinoid syndrome
• Cheiroarthropathy due to diabetes mellitus
• Eosinophilia myalgia syndrome
• Eosinophilic fasciitis
• Erythema migrans
• Fixed drug eruption
• Graft versus host disease
• Inflammatory granuloma annulare
• Interstitial and granulomatous dermatitis
• Interstitial mycosis fungoides
• Keloid and hypertrophic scar
• Lichen sclerosus et atrophicus
• Linear atrophoderma of Moulin
• Linear lupus erythematosus panniculitis
• Linear melorheostosis
• Lipodermatosclerosis
• Morpheaform dermatofibrosarcoma protuberans
• Muckle-Wells syndrome
• Nephrogenic fibrosing dermopathy
• Niemann-Pick disease
• Phenylketonuria
• POEMS syndrome
• Porphyria cutanea tarda
• Primary systemic amyloidosis
• Progeria
• Radiation fibrosis
• Reflex sympathetic dystrophy
• Restrictive dermopathy
• Scleroderma
• Sclerodermoid conditions caused by chemical/toxin exposures

  Polyvinyl chloride

  Epoxy resins

  Pesticides

  Dry cleaning solvents

  Silica dust

• Sclerodermoid conditions caused by iatrogenic agents

  Bleomycin

  Gemcitabine

  L-Tryptophan

  Melphalan isolated limb perfusion

  Pentazocine injections

  Silicone or paraffin implants

  Taxanes

  Uracil-Tegafur

  Vitamin K injections

• Scleromyxedema
• Stiff skin syndrome
• Sweet syndrome (early)
• Werner syndrome
• Winchester syndrome

Physical examination

Gallery

Trunk

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  Localized morphoea.

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  Localized morphoea.

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  Nodular morphoea.

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  Nodular morphoea.

Extremities

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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.

Additional Resources

• Aberer E, Neumann R, Stanek G. Is localised scleroderma a Borrelia infection? Lancet. Aug 3 1985;2(8449):278.

• Cunningham BB, Landells ID, Langman C, et al. Topical calcipotriene for morphea/linear scleroderma. J Am Acad Dermatol. Aug 1998;39(2 Pt 1):211-5.

• Daoud MS, Su WP, Leiferman KM, Perniciaro C. Bullous morphea: clinical, pathologic, and immunopathologic evaluation of thirteen cases. J Am Acad Dermatol. Jun 1994;30(6):937-43.

• Davis DA, Cohen PR, McNeese MD, Duvic M. Localized scleroderma in breast cancer patients treated with supervoltage external beam radiation: radiation port scleroderma. J Am Acad Dermatol. Dec 1996; 35 (6): 923-7.

• Dutz J. Treatment options for localized scleroderma. Skin Therapy Lett. 2000; 5 (2): 3-5.

• Dytoc M, Ting PT, Man J, et al. First case series on the use of imiquimod for morphoea. Br J Dermatol. Oct 2005;153(4):815-820.

• Eisen D, Alster TS. Use of a 585 nm pulsed dye laser for the treatment of morphea. Dermatol Surg. Jul 2002;28(7):615-6.

• Elst EF, Van Suijlekom-Smit LW, Oranje AP. Treatment of linear scleroderma with oral 1,25-dihydroxyvitamin D3 (calcitriol) in seven children. Pediatr Dermatol. Jan-Feb 1999;16(1):53-8.

• El-Azhary RA, Aponte CC, Nelson AM. Do antihistone autoantibodies reflect disease activity in linear scleroderma?. Arch Dermatol. Jun 2004;140(6):759-60.

• Falanga V, Medsger TA Jr, Reichlin M, Rodnan GP. Linear scleroderma. Clinical spectrum, prognosis, and laboratory abnormalities. Ann Intern Med. Jun 1986;104(6):849-57.

• Falanga V, Medsger TA Jr. D-penicillamine in the treatment of localized scleroderma. Arch Dermatol. May 1990;126(5):609-12.

• Falanga V, Medsger TA Jr, Reichlin M. Antinuclear and anti-single-stranded DNA antibodies in morphea and generalized morphea. Arch Dermatol. Mar 1987;123(3):350-3.

• Fitch PG, Rettig P, Burnham JM, et al. Treatment of pediatric localized scleroderma with methotrexate. J Rheumatol. Mar 2006;33(3):609-14.

• Fujiwara H, Fujiwara K, Hashimoto K, et al. Detection of Borrelia burgdorferi DNA (B garinii or B afzelii) in morphea and lichen sclerosus et atrophicus tissues of German and Japanese but not of US patients. Arch Dermatol. Jan 1997;133(1):41-4.

• Hayakawa I, Hasegawa M, Takehara K, Sato S. Anti-DNA topoisomerase IIalpha autoantibodies in localized scleroderma. Arthritis Rheum. Jan 2004;50(1):227-32.

• Holland KE, Steffes B, Nocton JJ, et al. Linear scleroderma en coup de sabre with associated neurologic abnormalities. Pediatrics. Jan 2006;117(1):e132-6.

• Hulshof MM, Bouwes Bavinck JN, Bergman W, et al. Double-blind, placebo-controlled study of oral calcitriol for the treatment of localized and systemic scleroderma. J Am Acad Dermatol. Dec 2000;43(6):1017-23.

• Igarashi A, Nashiro K, Kikuchi K, et al. Connective tissue growth factor gene expression in tissue sections from localized scleroderma, keloid, and other fibrotic skin disorders. J Invest Dermatol. Apr 1996;106(4):729-33.

• Ihn H, Fujimoto M, Sato S, et al. Increased levels of circulating intercellular adhesion molecule-1 in patients with localized scleroderma. J Am Acad Dermatol. Oct 1994;31(4):591-5.

• Ihn H, Sato S, Fujimoto M, et al. Clinical significance of serum levels of soluble interleukin-2 receptor in patients with * localized scleroderma. Br J Dermatol. May 1996;134(5):843-7.

• Itin PH, Schiller P. Double-lined frontoparietal scleroderma en coup de sabre. Dermatology. 1999;199(2):185-6.

• Jablonska S, Blaszczyk M. Is superficial morphea synonymous with atrophoderma Pasini-Pierini?. J Am Acad Dermatol. Jun 2004;50(6):979-80; author reply 980.

• Karrer S, Abels C, Landthaler M, Szeimies RM. Topical photodynamic therapy for localized scleroderma. Acta Derm Venereol. Jan-Feb 2000;80(1):26-7.

• Kencka D, Blaszczyk M, Jablonska S. Atrophoderma Pasini-Pierini is a primary atrophic abortive morphea. Dermatology. 1995;190(3):203-6.

• Kerscher M, Meurer M, Sander C, et al. PUVA bath photochemotherapy for localized scleroderma. Evaluation of 17 consecutive patients. Arch Dermatol. Nov 1996;132(11):1280-2.

• Kerscher M, Volkenandt M, Gruss C, et al. Low-dose UVA phototherapy for treatment of localized scleroderma. J Am Acad Dermatol. Jan 1998;38(1):21-6.

• Kikuchi K, Kadono T, Ihn H, et al. Growth regulation in scleroderma fibroblasts: increased response to transforming growth factor-beta 1. J Invest Dermatol. Jul 1995;105(1):128-32.

• Koeger AC, Rozenberg S, Fautrel B. Effectiveness of topical calcitriol for localized scleroderma. J Rheumatol. Jan 1999;26(1):239-40.

• Kreuter A, Hyun J, Stücker M, et al. A randomized controlled study of low-dose UVA1, medium-dose UVA1, and narrowband UVB phototherapy in the treatment of localized scleroderma. J Am Acad Dermatol. Mar 2006;54(3):440-7.

• Kreuter A, Gambichler T, Breuckmann F, et al. Pulsed high-dose corticosteroids combined with low-dose methotrexate in severe localized scleroderma. Arch Dermatol. Jul 2005;141(7):847-52.

• Kreuter A, Gambichler T, Avermaete A, et al. Combined treatment with calcipotriol ointment and low-dose ultraviolet A1 phototherapy in childhood morphea. Pediatr Dermatol. May-Jun 2001;18(3):241-5.

• Leask A, Denton CP, Abraham DJ. Insights into the molecular mechanism of chronic fibrosis: the role of connective tissue growth factor in scleroderma. J Invest Dermatol. 2004;122:1-6.

• Mancuso G, Berdondini RM. Localized scleroderma: response to occlusive treatment with tacrolimus ointment. Br J Dermatol. Jan 2005;152(1):180-2.

• Martin L, Piette F, Blanc P, et al. Clinical variants of the preprotuberant stage of dermatofibrosarcoma protuberans. Br J Dermatol. Nov 2005;153(5):932-6.

• Marzano AV, Menni S, Parodi A, et al. Localized scleroderma in adults and children. Clinical and laboratory investigations on 239 cases. Eur J Dermatol. Mar-Apr 2003;13(2):171-6.

• McKenna DB, Benton EC. A tri-linear pattern of scleroderma en coup de sabre following Blaschkos lines. Clin Exp Dermatol. Nov 1999;24(6):467-8.

• McNiff JM, Glusac EJ, Lazova RZ, Carroll CB. Morphea limited to the superficial reticular dermis: an underrecognized histologic phenomenon. Am J Dermatopathol. Aug 1999;21(4):315-9.

• Mohrenschlager M, Jung C, Ring J, Abeck D. Effect of penicillin G on corium thickness in linear morphea of childhood: An analysis using ultrasound technique. Pediatr Dermatol. Jul-Aug 1999;16(4):314-6.

• Morison WL. Psoralen UVA therapy for linear and generalized morphea. J Am Acad Dermatol. Oct 1997;37(4):657-9.

• Nagai M, Hasegawa M, Takehara K, Sato S. Novel autoantibody to Cu/Zn superoxide dismutase in patients with localized scleroderma. J Invest Dermatol. Mar 2004;122(3):594-601.

• Nelson AM. Localized scleroderma including morphea, linear scleroderma, and eosinophilic fasciitis. Curr Probl Pediatr. Oct 1996;26(9):318-24.

• Peterson LS, Nelson AM, Su WP. Classification of morphea (localized scleroderma). Mayo Clin Proc. Nov 1995;70(11):1068-76.

• Peterson LS, Nelson AM, Su WP, et al. The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993. J Rheumatol. Jan 1997;24(1):73-80.

• Rosenberg AM, Uziel Y, Krafchik BR, et al. Antinuclear antibodies in children with localized scleroderma. J Rheumatol. Dec 1995;22(12):2337-43.

• Ruffatti A, Peserico A, Rondinone R, et al. Prevalence and characteristics of anti-single-stranded DNA antibodies in localized scleroderma. Comparison with systemic lupus erythematosus. Arch Dermatol. Aug 1991;127(8):1180-3.

• Sapadin AN, Fleischmajer R. Treatment of scleroderma. Arch Dermatol. Jan 2002;138(1):99-105.

• Schaffer JV, Carroll C, Dvoretsky I, et al. Postirradiation morphea of the breast presentation of two cases and review of the literature. Dermatology. 2000;200(1):67-71.

• Seyger MM, van den Hoogen FH, de Boo T, de Jong EM. Low-dose methotrexate in the treatment of widespread morphea. J Am Acad Dermatol. Aug 1998;39(2 Pt 1):220-5.

• Shono S, Imura M, Ota M, et al. Lichen sclerosus et atrophicus, morphea, and coexistence of both diseases. Histological studies using lectins. Arch Dermatol. Sep 1991;127(9):1352-6.

• Soma Y, Fujimoto M. Frontoparietal scleroderma (en coup de sabre) following Blaschkos lines. J Am Acad Dermatol. Feb 1998;38(2 Pt 2):366-8.

• Sommer A, Gambichler T, Bacharach-Buhles M, et al. Clinical and serological characteristics of progressive facial hemiatrophy: a case series of 12 patients. J Am Acad Dermatol. Feb 2006;54(2):227-33.

• Stege H, Berneburg M, Humke S, et al. High-dose UVA1 radiation therapy for localized scleroderma. J Am Acad Dermatol. Jun 1997;36(6 Pt 1):938-44.

• Takehara K, Sato S. Localized scleroderma is an autoimmune disorder. Rheumatology (Oxford). Mar 2005;44(3):274-9.

• Uziel Y, Krafchik BR, Silverman ED, et al. Localized scleroderma in childhood: a report of 30 cases. Semin Arthritis Rheum. Apr 1994;23(5):328-40.

• Uziel Y, Feldman BM, Krafchik BR, et al. Methotrexate and corticosteroid therapy for pediatric localized scleroderma. J Pediatr. Jan 2000;136(1):91-5.

• Vierra E, Cunningham BB. Morphea and localized scleroderma in children. Semin Cutan Med Surg. Sep 1999;18(3):210-25.

• Weide B, Walz T, Garbe C. Is morphoea caused by Borrelia burgdorferi? A review. Br J Dermatol. Apr 2000;142(4):636-44.

• Weide B, Schittek B, Klyscz T, et al. Morphoea is neither associated with features of Borrelia burgdorferi infection, nor is this agent detectable in lesional skin by polymerase chain reaction. Br J Dermatol. Oct 2000;143(4):780-5.

• Wienecke R, Schlupen EM, Zochling N, et al. No evidence for Borrelia burgdorferi-specific DNA in lesions of localized scleroderma. J Invest Dermatol. Jan 1995;104(1):23-6.

• Wollina U, Looks A, Uhlemann C, Wollina K. Pansclerotic morphea of childhood-follow-up over 6 years. Pediatr Dermatol. May-Jun 1999;16(3):245-7.

• Zulian F, Vallongo C, Woo P, et al. Localized scleroderma in childhood is not just a skin disease. Arthritis Rheum. Sep 2005;52(9):2873-81.

• Zulian F, Athreya BH, Laxer R, et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford). Dec 20 2005;

External links

• UT-Southwestern Medical Center at Dallas Morphea Registry
• Scleroderma Foundation
• Scleroderma Society of Ontario
• International Scleroderma Network
• The Scleroderma Research Foundation
• UK Scleroderma Society

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