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.[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]

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

Skin

Trunk

Extremities

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. Morpea CNN.com, (May 05, 2006).
  2. Peterson LS, Nelson AM, Su WP (1995). "Classification of morphea (localized scleroderma)". Mayo Clin. Proc. 70 (11): 1068–76. PMID 7475336. 
  3. 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. PMID 16368732. doi:10.1093/rheumatology/kei251. 
  4. 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. 
  5. Laxer RM, Zulian F (2006). "Localized scleroderma". Curr Opin Rheumatol. 18 (6): 606–13. PMID 17053506. doi:10.1097/01.bor.0000245727.40630.c3. 
  6. Hayakawa I, Hasegawa M, Takehara K, Sato S (2004). "Anti-DNA topoisomerase IIalpha autoantibodies in localized scleroderma". Arthritis Rheum. 50 (1): 227–32. PMID 14730620. doi:10.1002/art.11432. 
  7. 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. PMID 11123778. doi:10.1046/j.1525-1470.2000.01820.x. 
  8. 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. PMID 16405610. doi:10.1111/j.1468-3083.2005.01336.x. 
  9. 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. PMID 17040502. doi:10.1111/j.1346-8138.2006.00165.x. 

Additional Resources

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