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Summary

Morphea is an uncommon but potentially disabling condition. Abnormalities in immune system parameters, endothelial activation and fibroblast metabolism have been described, but a unifying pathophysiologic model remains to be tested. The clinician is faced with considerable uncertainty when choosing a treatment modality for LS. Given the benign natural progression of plaque type morphea, treatment with topical modalities such as superpotent corticosteroids or calcipotriol is prudent. For more generalized forms of morphea, as well as the linear forms, UVA is currently the best-documented therapeutic modality. In the absence of access to UVA treatment, oral calciferol or systemic immunosuppression may be contemplated. Recent studies support the use of methotrexate with systemic corticosteroids for the management of aggressive disease. The authors have had variable success with PUVA, broadband UVA, and methotrexate in severe disease. Resolution of therapeutic uncertainty must await the organization and completion of controlled trials.

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References

  • Aberer E, Neumann R, Stanek G (1985) Is localised scleroderma a borrelia infection? [letter]. Lancet 2(8449):278

    PubMed  CAS  Google Scholar 

  • Aiba S, Tabata N, Ohtani H, Tagami H (1994) Cd34+ spindle-shaped cells selectively disappear from the skin lesion of scleroderma. Arch Dermatol 130(5):593–597

    PubMed  CAS  Google Scholar 

  • Akimoto S, Hayashi H, Ishikawa H (1992) Disaccharide analysis of the skin glycosaminoglycans in systemic sclerosis. Br J Dermatol 126(1):29–34

    PubMed  CAS  Google Scholar 

  • Andres C, Kollmar A, Mempel M, Hein R, Ring J, Eberlein B (2010) Successful ultraviolet a1 phototherapy in the treatment of localized scleroderma: A retrospective and prospective study. Br J Dermatol 162(2):445–447

    PubMed  CAS  Google Scholar 

  • Antiga E, Quaglino P, Bellandi S, Volpi W, Del Bianco E, Comessatti A, Osella-Abate S, De Simone C, Marzano A, Bernengo MG, Fabbri P, Caproni M (2010) Regulatory T cells in the skin lesions and blood of patients with systemic sclerosis and morphoea. Br J Dermatol 162(5):1056–1063

    PubMed  CAS  Google Scholar 

  • Arkachaisri T, Fertig N, Pino S, Medsger TA, Jr. (2008) Serum autoantibodies and their clinical associations in patients with childhood-and adult-onset linear scleroderma. A single-center study. J Rheumatol 35(12):2439–2444

    PubMed  CAS  Google Scholar 

  • Arkachaisri T, Vilaiyuk S, Torok KS, Medsger TA, Jr. (2010) Development and initial validation of the localized scleroderma skin damage index and physician global assessment of disease damage: A proof-of-concept study. Rheumatology (Oxford) 49(2):373–381

    Google Scholar 

  • Arnett FC, Tan FK, Uziel Y, Laxer RM, Krafchik BR, Antohi S, Bona C (1999) Autoantibodies to the extracellular matrix microfibrillar protein, fibrillin 1, in patients with localized scleroderma. Arthritis Rheum 42(12):2656–2659

    PubMed  CAS  Google Scholar 

  • Balat A, Akinci A, Turgut M, Mizrak B, Aydin A (1999) Eosinophilic fasciitis — progression to linear scleroderma: A case report. Turk J Pediatr 41(3):381–385

    PubMed  CAS  Google Scholar 

  • Barrera P, Haagsma CJ, Boerbooms AM, Van Riel PL, Borm GF, Van de Putte LB, Van der Meer JW (1995) Effect of methotrexate alone or in combination with sulphasalazine on the production and circulating concentrations of cytokines and their antagonists. Longitudinal evaluation in patients with rheumatoid arthritis. Br J Rheumatol 34(8):747–755

    PubMed  CAS  Google Scholar 

  • Batchelor R, Lamb S, Goulden V, Stables G, Goodfield M, Merchant W (2008) Photodynamic therapy for the treatment of morphoea. Clin Exp Dermatol 33(5):661–663

    PubMed  CAS  Google Scholar 

  • Bilen N, Apaydin R, Ercin C, Harova G, Basdas F, Bayramgurler D (1999) Coexistence of morphea and psoriasis responding to acitretin treatment. J Eur Acad Dermatol Venereol 13(2):113–117

    PubMed  CAS  Google Scholar 

  • Birdi N, Shore A, Rush P, Laxer RM, Silverman ED, Krafchik B (1992) Childhood linear scleroderma: A possible role of thermography for evaluation. J Rheumatol 19(6):968–973

    PubMed  CAS  Google Scholar 

  • Blaszczyk M, Jablonska S (1999) Linear scleroderma en coup de sabre. Relationship with progressive facial hemiatrophy (pfh). In: Uitto Ma (ed) Rheumaderm. Kluwer Academic / Plenum Publishers, New York, pp 101–104

    Google Scholar 

  • Blaszczyk M, Krolicki L, Krasu M, Glinska O, Jablonska S (2003) Progressive facial hemiatrophy: Central nervous system involvement and relationship with scleroderma en coup de sabre. J Rheumatol 30(9): 1997–2004

    PubMed  Google Scholar 

  • Blaszczyk M, Krysicka-Janiger K, Jablonska S (2000) Primary atrophic profound linear scleroderma. Report of three cases. Dermatology 200(1):63–66

    PubMed  CAS  Google Scholar 

  • Bleasel NR, Stapleton KM, Commens C, Ahern VA (1999) Radiation-induced localized scleroderma in breast cancer patients. Australas J Dermatol 40(2):99–102

    PubMed  CAS  Google Scholar 

  • Bodemer C, Amoric JC, Hamel-Teillac D, De Prost Y (1999a) Localized scleroderma in children and a therapeutic trial using calcitriol: A therapeutic possibility to define. Ann Dermatol Venereol 126(10):725–726

    PubMed  CAS  Google Scholar 

  • Bodemer C, Belon M, Hamel-Teillac D, Amoric JC, Fraitag S, Prieur AM, De Prost Y (1999b) [scleroderma in children: A retrospective study of 70 cases]. Ann Dermatol Venereol 126(10):691–694

    PubMed  CAS  Google Scholar 

  • Boelsma E, Pavel S, Ponec M (1995) Effects of calcitriol on fibroblasts derived from skin of scleroderma patients. Dermatology 191(3):226–233

    PubMed  CAS  Google Scholar 

  • Bottomley WW, Jutley J, Wood EJ, Goodfield MD (1995) The effect of calcipotriol on lesional fibroblasts from patients with active morphoea. Acta Derm Venereol 75(5):364–366

    PubMed  CAS  Google Scholar 

  • Branchet MC, Boisnic S, Bletry O, Robert L, Charron D, Frances C (1992) Expression of hla class ii antigens on skin fibroblasts in scleroderma. Br J Dermatol 126(5):431–435

    PubMed  CAS  Google Scholar 

  • Brownell I, Soter NA, Franks AG, Jr. (2007) Familial linear scleroderma (en coup de sabre) responsive to antimalarials and narrowband ultraviolet b therapy. Dermatology online journal 13(1):11

    PubMed  Google Scholar 

  • Caca-Biljanovska NG, Vlckova-Laskoska MT, Dervendi DV, Pesic NP, Laskoski DS (1999) Treatment of generalized morphea with oral 1,25-dihydroxyvitamin d3. Adv Exp Med Biol 455:299–304

    PubMed  CAS  Google Scholar 

  • Camacho NR, Sanchez JE, Martin RF, Gonzalez JR, Sanchez JL (2001) Medium-dose uva1 phototherapy in localized scleroderma and its effect in cd34-positive dendritic cells. J Am Acad Dermatol 45(5):697–699

    PubMed  CAS  Google Scholar 

  • Campione E, Paterno EJ, Diluvio L, Orlandi A, Bianchi L, Chimenti S (2009) Localized morphea treated with imiquimod 5% and dermoscopic assessment of effectiveness. The Journal of dermatological treatment 20(1):10–13

    PubMed  CAS  Google Scholar 

  • Canizares O, Sachs P, Jaimovich L, Torres V (1958) Idiopathic atrophoderma of pasini and pierini. Arch Dermatol 77:42–59

    CAS  Google Scholar 

  • Cannick L, 3rd, Douglas G, Crater S, Silver R (2003) Nodular scleroderma: Case report and literature review. J Rheumatol 30(11):2500–2502

    PubMed  Google Scholar 

  • Castanet J, Lacour JP, Perrin C, Taillan B, Dubois D, Ortonne JP (1994) Association of eosinophilic fasciitis, multiple morphea and antiphospholipid antibody. Dermatology 189(3):304–307

    PubMed  CAS  Google Scholar 

  • Christianson H, Dorsey C, O’Leary P, Kierland R (1956) Localized scleroderma: A clinical study of twohundred thirty-five cases. Arch Dermatol 74:629–639

    CAS  Google Scholar 

  • Chung H, Hann S (1997) Lupus panniculitis treated by a combination therapy of hydroxycvhloroquine and quinacrine. J Dermatol 24(9):569–572

    PubMed  CAS  Google Scholar 

  • Chung MH, Sum J, Morrell MJ, Horoupian DS (1995) Intracerebral involvement in scleroderma en coup de sabre: Report of a case with neuropathologic findings. Ann Neurol 37(5):679–681

    PubMed  CAS  Google Scholar 

  • Clemens TL, Adams JS, Horiuchi N, Gilchrest BA, Cho H, Tsuchiya Y, Matsuo N, Suda T, Holick MF (1983) Interaction of 1,25-dihydroxyvitamin-d3 with keratinocytes and fibroblasts from skin of normal subjects and a subject with vitamin-d-dependent rickets, type ii: A model for study of the mode of action of 1,25-dihydroxyvitamin d3. J Clin Endocrinol Metab 56(4):824–830

    PubMed  CAS  Google Scholar 

  • Cosnes A, Anglade MC, Revuz J, Radier C (2003) Thirteen-megahertz ultrasound probe: Its role in diagnosing localized scleroderma. Br J Dermatol 148(4):724–729

    PubMed  CAS  Google Scholar 

  • Crespo MP, Mas IB, Diaz JM, Costa AL, Nortes IB (2009) Rapid response to cyclosporine and maintenance with methotrexate in linear scleroderma in a young girl. Pediatr Dermatol 26(1):118–120

    PubMed  Google Scholar 

  • Cribier B, Faradji T, Le Coz C, Oberling F, Grosshans E (1995) Extracorporeal photochemotherapy in systemic sclerosis and severe morphea. Dermatology 191(1):25–31

    PubMed  CAS  Google Scholar 

  • Cunningham BB, Landells ID, Langman C, Sailer DE, Paller AS (1998) Topical calcipotriene for morphea/linear scleroderma. J Am Acad Dermatol 39 (2 Pt 1):211–215

    PubMed  CAS  Google Scholar 

  • Curley RK, Macfarlane AW, Evans S, Woodrow JC (1987) The treatment of linear morphoea with d-penicillamine. Clin Exp Dermatol 12(1):56–57

    PubMed  CAS  Google Scholar 

  • Czarnecki DB, Taft EH (1982) Generalized morphoea successfully treated with salazopyrine. Acta Derm Venereol 62(1):81–82

    PubMed  CAS  Google Scholar 

  • Daoud MS, Su WP, Leiferman KM, Perniciaro C (1994) Bullous morphea: Clinical, pathologic, and immunopathologic evaluation of thirteen cases. J Am Acad Dermatol 30(6):937–943

    PubMed  CAS  Google Scholar 

  • David J, Wilson J, Woo P (1991) Scleroderma “En coup de sabre”. Ann Rheum Dis 50:260–262

    PubMed  CAS  Google Scholar 

  • de Gannes GC, Ghoreishi M, Pope J, Russell A, Bell D, Adams S, Shojania K, Martinka M, Dutz JP (2007) Psoriasis and pustular dermatitis triggered by tnf-{alpha} inhibitors in patients with rheumatologic conditions. Arch Dermatol 143(2):223–231

    PubMed  Google Scholar 

  • Dehen L, Roujeau JC, Cosnes A, Revuz J (1994) Internal involvement in localized scleroderma. Medicine (Baltimore) 73(5):241–245

    CAS  Google Scholar 

  • Diaz-Perez J, Connolly S, Winkelmann R (1980) Disabling pansclerotic morphea of children. Arch Dermatol 116:169–173

    PubMed  CAS  Google Scholar 

  • Dillon WI, Saed GM, Fivenson DP (1995) Borrelia burgdorferi DNA is undetectable by polymerase chain reaction in skin lesions of morphea, scleroderma, or lichen sclerosus et atrophicus of patients from north america. J Am Acad Dermatol 33(4):617–620

    PubMed  CAS  Google Scholar 

  • Doyle JA, Ginsburg WW (1989) Eosinophilic fasciitis. Med Clin North Am 73(5):1157–1166

    PubMed  CAS  Google Scholar 

  • Drago F, Rampini P, Lugani C, Rebora A (1998) Generalized morphoea after antitetanus vaccination [letter]. Clin Exp Dermatol 23(3): 142

    PubMed  CAS  Google Scholar 

  • Dubois EL, Chandor S, Friou GJ, Bischel M (1971) Progressive systemic sclerosis (pss) and localized scleroderma (morphea) with positive le cell test and unusual systemic manifestations compatible with systemic lupus erythematous (sle): Presentation of 14 cases including one set of identical twins, one with scleroderma dn the other with sle. Review of the literature. Medicine (Baltimore) 50(3):199–222

    CAS  Google Scholar 

  • Dytoc M, Ting PT, Man J, Sawyer D, Fiorillo L (2005) First case series on the use of imiquimod for morphoea. Br J Dermatol 153(4):815–820

    PubMed  CAS  Google Scholar 

  • Dytoc MT, Kossintseva I, Ting PT (2007) First case series on the use of calcipotriol-betamethasone dipropionate for morphoea. Br J Dermatol 157(3):615–618

    PubMed  CAS  Google Scholar 

  • Eisen D, Alster TS (2002) Use of a 585 nm pulsed dye laser for the treatment of morphea. Dermatol Surg 28(7):615–616

    PubMed  Google Scholar 

  • El-Mofty M, Mostafa W, El-Darouty M, Bosseila M, Nada H, Yousef R, Esmat S, El-Lawindy M, Assaf M, El-Enani G (2004) Different low doses of broad-band uva in the treatment of morphea and systemic sclerosis. Photodermatol Photoimmunol Photomed 20(3): 148–156

    PubMed  CAS  Google Scholar 

  • El-Mofty M, Zaher H, Bosseila M, Yousef R, Saad B (2000) Low-dose broad-band uva in morphea using a new method for evaluation. Photodermatol Photoimmunol Photomed 16(2):43–49

    PubMed  CAS  Google Scholar 

  • Elst EF, Van Suijlekom-Smit LW, Oranje AP (1999) Treatment of linear scleroderma with oral 1,25-dihydroxyvitamin d3 (calcitriol) in seven children. Pediatr Dermatol 16(1):53–58

    PubMed  CAS  Google Scholar 

  • Falanga V, Medsger TA, Jr. (1990) D-penicillamine in the treatment of localized scleroderma. Archives of Dermatology 126(5):609–612

    PubMed  CAS  Google Scholar 

  • Falanga V, Medsger TA, Jr., Reichlin M (1987) Antinuclear and anti-single-stranded DNA antibodies in morphea and generalized morphea. Arch Dermatol 123(3):350–353

    PubMed  CAS  Google Scholar 

  • Falanga V, Medsger TA, Jr., Reichlin M, Rodnan GP (1986) Linear scleroderma. Clinical spectrum, prognosis, and laboratory abnormalities. Ann Intern Med 104(6):849–857

    PubMed  CAS  Google Scholar 

  • Falanga V, Medsger TA, Reichlin M (1985) High titers of antibodies to single-stranded DNA in linear scleroderma. Arch Dermatol 121(3):345–347

    PubMed  CAS  Google Scholar 

  • Fleischmajer R, Nedwich A (1972) Generalized morphea. I. Histology of the dermis and subcutaneous tissue. Arch Dermatol 106(4):509–514

    PubMed  CAS  Google Scholar 

  • Fleischmajer R, Perlish JS (1972) Glycosaminoglycans in scleroderma and scleredema. J Invest Dermatol 58(3):129–132

    PubMed  CAS  Google Scholar 

  • Fleischmajer R, Prunieras M (1972) Generalized morphea. II. Electron microscopy of collagen, cells, and the subcutaneous tissue. Arch Dermatol 106(4):515–524

    PubMed  CAS  Google Scholar 

  • Fleischmajer R, Perlish JS, Krieg T, Timpl R (1981) Variability in collagen and fibronectin synthesis by scleroderma fibroblasts in primary culture. J Invest Dermatol 76(5):400–403

    PubMed  CAS  Google Scholar 

  • Foeldvari I (1998) Progressive linear scleroderma and morphea in a child. J Pediatr 133(2):308

    PubMed  CAS  Google Scholar 

  • Fry A, Alvarellos A, Fink C, Blaw M, Roach E (1992) Intracranial findings in progressive facial hemiatrophy. J Rheumatol 19:956–958

    PubMed  CAS  Google Scholar 

  • Fujimoto M, Sato S, Ihn H, Takehara K (1995) Autoantibodies to the heat-shock protein hsp73 in localized scleroderma. Arch Dermatol Res 287(6):581–585

    PubMed  CAS  Google Scholar 

  • Gambichler T, Kreuter A, Rotterdam S, Altmeyer P, Hoffmann K (2003) Linear scleroderma ‘en coup de sabre’ treated with topical calcipotriol and cream psoralen plus ultraviolet a. J Eur Acad Dermatol Venereol 17(5):601–602

    PubMed  CAS  Google Scholar 

  • Garcia-Bustinduy M, Noda A, Sanchez R, Gonzalez de Mesa MJ, Guimera F, Garcia-Montelongo R (1998) Puva therapy in localized scleroderma [letter]. J Eur Acad Dermatol Venereol 10(3):283–284

    PubMed  CAS  Google Scholar 

  • Garzon M, Paller A (1999) Ultrapotent topical corticosteroids treatment of childhood genital lichen sclerosus. Arch Dermatol 135(5):525–528

    PubMed  CAS  Google Scholar 

  • Gordon GV (1981) Eosinophilic fasciitis. A case report and review of the literature. Cutis 28(3):268, 271–263

    PubMed  CAS  Google Scholar 

  • Grundmann-Kollmann M, Ochsendorf F, Zollner TM, Spieth K, Sachsenberg-Studer E, Kaufmann R, Podda M (2000) Puva-cream photochemotherapy for the treatment of localized scleroderma. J Am Acad Dermatol 43(4):675–678

    PubMed  CAS  Google Scholar 

  • Gruss C, Reed JA, Altmeyer P, McNutt NS, Kerscher M (1997a) Induction of interstitial collagenase (mmp-1) by uva-1 phototherapy in morphea fibroblasts. Lancet 350(9087): 1295–1296

    PubMed  CAS  Google Scholar 

  • Gruss C, Reed JA, Altmeyer P, McNutt NS, Kerscher M (1997b) Induction of interstitial collagenase (mmp-1) by uva-1 phototherapy in morphea fibroblasts [letter]. Lancet 350(9087):1295–1296

    PubMed  CAS  Google Scholar 

  • Gruss C, Stucker M, Kobyletzki G, Schreiber D, Altmeyer P, Kerscher M (1997c) Low dose uva1 phototherapy in disabling pansclerotic morphoea of childhood [letter]. Br J Dermatol 136(2):293–294

    PubMed  CAS  Google Scholar 

  • Hasegawa M, Sato S, Nagaoka T, Fujimoto M, Takehara K (2003) Serum levels of tumor necrosis factor and interleukin-13 are elevated in patients with localized scleroderma. Dermatology 207(2):141–147

    PubMed  CAS  Google Scholar 

  • Hatamochi A, Ono M, Arakawa M, Takeda K, Ueki H (1992) Analysis of collagen gene expression by cultured fibroblasts in morphoea. Br J Dermatol 126(3):216–221

    PubMed  CAS  Google Scholar 

  • Hauser C, Skaria A, Harms M, Saurat JH (1996) Morphoea following blaschko’s lines [letter]. Br J Dermatol 134(3):594–595

    PubMed  CAS  Google Scholar 

  • Hayakawa I, Hasegawa M, Takehara K, Sato S (2004) Anti-DNA topoisomerase iialpha autoanti-bodies in localized scleroderma. Arthritis Rheum 50(1):227–232

    PubMed  CAS  Google Scholar 

  • Herrick AL, Ennis H, Bhushan M, Silman AJ, Baildam EM (2010) Incidence of childhood linear scleroderma and systemic sclerosis in the uk and ireland. Arthritis Care Res (Hoboken) 62(2):213–218

    Google Scholar 

  • Higley H, Persichitte K, Chu S, Waegell W, Vancheeswaran R, Black C (1994) Immunocytochemical localization and serologic detection of transforming growth factor beta 1. Association with type i procollagen and inflammatory cell markers in diffuse and limited systemic sclerosis, morphea, and raynaud’s phenomenon. Arthritis Rheum 37(2):278–288

    PubMed  CAS  Google Scholar 

  • Holick MF, Smith E, Pincus S (1987) Skin as the site of vitamin d synthesis and target tissue for 1,25-dihydroxyvitamin d3. Use of calcitriol (1,25-dihydroxyvitamin d3) for treatment of psoriasis. Arch Dermatol 123(12):1677–1683a

    PubMed  CAS  Google Scholar 

  • Hsu S, Lee MW, Carlton S, Kramer EM (1999) Nodular morphea in a linear pattern. Int J Dermatol 38(7):529–530

    PubMed  CAS  Google Scholar 

  • Hulshof MM, Pavel S, Breedveld FC, Dijkmans BA, Vermeer BJ (1994) Oral calcitriol as a new therapeutic modality for generalized morphea. Arch Dermatol 130(10):1290–1293

    PubMed  CAS  Google Scholar 

  • Hulshof MM, Bouwes Bavinck JN, Bergman W, Masclee AA, Heickendorff L, Breedveld FC, Dijkmans BA (2000) Double-blind, placebo-controlled study of oral calcitriol for the treatment of localized and systemic scleroderma. J Am Acacd Dermatol 43(6):1017–1023

    CAS  Google Scholar 

  • Humbert P, Aubin F, Dupond JL, Delaporte E (1995) Oral calcitriol as a new therapeutic agent in localized and systemic scleroderma. Archives of Dermatology 131(7):850–851

    PubMed  CAS  Google Scholar 

  • Humbert PG, Dupond JL, Rochefort A, Vasselet R, Lucas A, Laurent R, Agache P (1990) Localized scleroderma — response to 1,25-dihydroxyvitamin d3. Clin Exp Dermatol 15(5):396–398

    PubMed  CAS  Google Scholar 

  • Hunzelmann N, Anders S, Fierlbeck G, Hein R, Herrmann K, Albrecht M, Bell S, Muche R, Wehner-Caroli J, Gaus W, Krieg T (1997) Double-blind, placebo-controlled study of intralesional interferon gamma for the treatment of localized scleroderma. J Am Acad Dermatol 36(3):433–435

    PubMed  CAS  Google Scholar 

  • Igarashi A, Nashiro K, Kikuchi K, Sato S, Ihn H, Fujimoto M, Grotendorst GR, Takehara K (1996) Connective tissue growth factor gene expression in tissue sections from localized scleroderma, keloid, and other fibrotic skin disorders. J Invest Dermatol 106(4):729–733

    PubMed  CAS  Google Scholar 

  • Ihn H, Sato S, Fujimoto M, Kikuchi K, Takehara K (1994) Demonstration of interleukin 8 in serum samples of patients with localized scleroderma [letter]. Arch Dermatol 130(10):1327–1328

    PubMed  CAS  Google Scholar 

  • Ihn H, Sato S, Fujimoto M, Kikuchi K, Takehara K (1995) Demonstration of interleukin-2, interleukin-4 and interleukin-6 in sera from patients with localized scleroderma. Arch Dermatol Res 287(2): 193–197

    PubMed  CAS  Google Scholar 

  • Ihn H, Sato S, Fujimoto M, Kikuchi K, Takehara K (1996) Clinical significance of serum levels of soluble interleukin-2 receptor in patients with localized scleroderma. Br J Dermatol 134(5):843–847

    PubMed  CAS  Google Scholar 

  • Ihn H, Yazawa N, Kubo M, Yamane K, Sato S, Fujimoto M, Kikuchi K, Soma Y, Tamaki K (2000) Circulating levels of soluble cd30 are increased in patients with localized scleroderma and correlated with serological and clinical features of the disease. J Rheumatol 27(3):698–702

    PubMed  CAS  Google Scholar 

  • Itin PH, Schiller P (1999) Double-lined frontoparietal scleroderma en coup de sabre. Dermatology 199(2):185–186

    PubMed  CAS  Google Scholar 

  • Jablonska S (1975a) Facial hemiatrophy and it’s relation to localized scleroderma. In: Jablonska S (ed) Scleroderma and pseudoscleroderma. PZWL, Warsaw, pp 537–548

    Google Scholar 

  • Jablonska S (1975b) Localised scleroderma. In: Jablonska S (ed) Scleroderma and pseudoscleroderma. 2nd edn. Polish Medical Publishers, Warsaw, pp 277–303

    Google Scholar 

  • Jablonska S, Rodnan G (1979) Localized forms of scleroderma. Clin Rheumatol Dis 5:215–241

    Google Scholar 

  • Jablonska S, Blaszczyk M (2004) Is superficial morphea synonymous with atrophoderma pasinipierini? J Am Acad Dermatol 50(6):979–980

    PubMed  Google Scholar 

  • Jacobe HT, Cayce R, Nguyen J (2008) Uva1 phototherapy is effective in darker skin: A review of 101 patients of fitzpatrick skin types i–v. Br J Dermatol 159(3):691–696

    PubMed  CAS  Google Scholar 

  • Janzen L, Jeffery JR, Gough J, Chalmers IM (1995) Response to methotrexate in a patient with idiopathic eosinophilic fasciitis, morphea, igm hypergammaglobulinemia, and renal involvement. J Rheumatol 22(10):1967–1970

    PubMed  CAS  Google Scholar 

  • Jaworsky C (1997) Connective tissue diseases. In: Elder D (ed) Lever’s histopathology of the skin. Lippincott-Raven, Philadelphia, pp 253–285

    Google Scholar 

  • Jeffery LE, Burke F, Mura M, Zheng Y, Qureshi OS, Hewison M, Walker LS, Lammas DA, Raza K, Sansom DM (2009) 1,25-dihydroxyvitamin d3 and il-2 combine to inhibit t cell production of inflammatory cytokines and promote development of regulatory t cells expressing ctla-4 and foxp3. J Immunol 183(9):5458–5467

    PubMed  CAS  Google Scholar 

  • Jessop S, Whitelaw D (2009) Methotrexate for sclerotic skin disorders: No evidence for effectiveness. Br J Dermatol 161(5):1205

    PubMed  CAS  Google Scholar 

  • Joly P, Bamberger N, Crickx B, Belaich S (1994) Treatment of severe forms of localized scleroderma with oral corticosteroids: Follow-up study on 17 patients. Archives of Dermatology 130(5):663–664

    PubMed  CAS  Google Scholar 

  • Jones SM, Mathew CM, Dixey J, Lovell CR, McHugh NJ (1996) Vcam-1 expression on endothelium in lesions from cutaneous lupus erythematosus is increased compared with systemic and localized scleroderma. Br J Dermatol 135(5):678–686

    PubMed  CAS  Google Scholar 

  • Kahari VM, Heino J, Vuorio T, Vuorio E (1988a) Interferon-alpha and interferon-gamma reduce excessive collagen synthesis and procollagen mrna levels of scleroderma fibroblasts in culture. Biochim Biophys Acta 968(1):45–50

    PubMed  CAS  Google Scholar 

  • Kahari VM, Sandberg M, Kalimo H, Vuorio T, Vuorio E (1988b) Identification of fibroblasts responsible for increased collagen production in localized scleroderma by in situ hybridization. J Invest Dermatol 90(5):664–670

    PubMed  CAS  Google Scholar 

  • Kakimoto CV, Victor Ross E, Uebelhoer NS (2009) En coup de sabre presenting as a port-wine stain previously treated with pulsed dye laser. Dermatol Surg 35(1):165–167

    PubMed  CAS  Google Scholar 

  • Kanekura T, Fukumaru S, Matsushita S, Terasaki K, Mizoguchi S, Kanzaki T (1996) Successful treatment of scleroderma with puva therapy. J Dermatol 23(7):455–459

    PubMed  CAS  Google Scholar 

  • Karrer S, Abels C, Landthaler M, Szeimies RM (2000) Topical photodynamic therapy for localized scleroderma. Acta Derm Venereol 80(1):26–27

    PubMed  CAS  Google Scholar 

  • Kencka D, Blaszczyk M, Jablonska S (1995) Atrophoderma pasini-pierini is a primary atrophic abortive morphea. Dermatology 190(3):203–206

    PubMed  CAS  Google Scholar 

  • Kerscher M, Dirschka T, Volkenandt M (1995a) Treatment of localised scleroderma by uva1 phototherapy. Lancet 346(8983): 1166

    PubMed  CAS  Google Scholar 

  • Kerscher M, Meurer M, Sander C, Volkenandt M, Lehmann P, Plewig G, Rocken M (1996) Puva bath photochemotherapy for localized scleroderma. Evaluation of 17 consecutive patients. Arch Dermatol 132(11):1280–1282

    PubMed  CAS  Google Scholar 

  • Kerscher M, Volkenandt M, Gruss C, Reuther T, von Kobyletzki G, Freitag M, Dirschka T, Altmeyer P (1998b) Low-dose uva phototherapy for treatment of localized scleroderma. J Am Acad Dermatol 38(1):21–26

    PubMed  CAS  Google Scholar 

  • Ko JY, Kim YS, Lee CW (2009) Multifocal lesions of morphoea in a patient with systemic lupus erythematosus. Clin Exp Dermatol 34(8):e676–e679

    PubMed  CAS  Google Scholar 

  • Kobayashi KA, Lui H, Prendiville JS (1991) Solitary morphea profunda in a 5-year-old girl: Case report and review of the literature. Pediatr Dermatol 8(4):292–295

    PubMed  CAS  Google Scholar 

  • Kobayasi T, Serup J (1985) Vascular changes in morphea. Acta Derm Venereol 65(2):116–120

    PubMed  CAS  Google Scholar 

  • Kobayasi T, Willeberg A, Serup J, Ullman S (1990) Generalized morphea with blisters. A case report. Acta Derm Venereol 70(5):454–456

    PubMed  CAS  Google Scholar 

  • Koeger AC, Rozenberg S, Fautrel B (1999) Effectiveness of topical calcitriol for localized scleroderma [letter]. J Rheumatol 26(1):239–240

    PubMed  CAS  Google Scholar 

  • Kowalewski C, Kozlowska A, Zawadzka M, Wozniak K, Blaszczyk M, Jablonska S (2004) Alterations of basement membrane zone in bullous and non-bullous variants of extragenital lichen sclerosus. Am J Dermatopathol 26(2):96–101

    PubMed  Google Scholar 

  • Krell JM, Solomon AR, Glavey CM, Lawley TJ (1995) Nodular scleroderma. J Am Acad Dermatol 32 (2 Pt 2):343–345

    PubMed  CAS  Google Scholar 

  • Kreuter A, Gambichler T, Avermaete A, Jansen T, Hoffmann M, Hoffmann K, Altmeyer P, von Kobyletzki G, Bacharach-Buhles M (2001) Combined treatment with calcipotriol ointment and low-dose ultraviolet a1 phototherapy in childhood morphea. Pediatr Dermatol 18(3):241–245

    PubMed  CAS  Google Scholar 

  • Kreuter A, Gambichler T, Breuckmann F, Rotterdam S, Freitag M, Stuecker M, Hoffmann K, Altmeyer P (2005) Pulsed high-dose corticosteroids combined with low-dose methotrexate in severe localized scleroderma. Arch Dermatol 141(7):847–852

    PubMed  CAS  Google Scholar 

  • Kreuter A, Hyun J, Skrygan M, Sommer A, Bastian A, Altmeyer P, Gambichler T (2006a) Ultraviolet a1-induced downregulation of human beta-defensins and interleukin-6 and interleukin-8 correlates with clinical improvement in localized scleroderma. Br J Dermatol 155(3):600–607

    PubMed  CAS  Google Scholar 

  • Kreuter A, Hyun J, Stucker M, Sommer A, Altmeyer P, Gambichler T (2006b) 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 54(3):440–447

    PubMed  Google Scholar 

  • Kroft EB, Creemers MC, van den Hoogen FH, Boezeman JB, de Jong EM (2009a) Effectiveness, side-effects and period of remission after treatment with methotrexate in localized scleroderma and related sclerotic skin diseases: An inception cohort study. Br J Dermatol 160(5):1075–1082

    PubMed  CAS  Google Scholar 

  • Kroft EB, de Jong EM, Evers AW (2009b) Psychological distress in patients with morphea and eosinophilic fasciitis. Arch Dermatol 145(9):1017–1022

    PubMed  Google Scholar 

  • Kubo M, Ihn H, Yamane K, Tamaki K (2001) Up-regulated expression of transforming growth factor beta receptors in dermal fibroblasts in skin sections from patients with localized scleroderma. Arthritis Rheum 44(3):731–734

    PubMed  CAS  Google Scholar 

  • Labandeira J, Leon-Mateos A, Suarez-Penaranda JM, Garea MT, Toribio J (2003) What is nodularkeloidal scleroderma? Dermatology 207(2):130–132

    PubMed  CAS  Google Scholar 

  • Lee HJ, Kim MY, Ha SJ, Kim JW (2002) Two cases of morphea associated with hashimoto’s thyroiditis. Acta Derm Venereol 82(1):58–59

    PubMed  Google Scholar 

  • Leitenberger JJ, Cayce RL, Haley RW, Adams-Huet B, Bergstresser PR, Jacobe HT (2009) Distinct autoimmune syndromes in morphea: A review of 245 adult and pediatric cases. Arch Dermatol 145(5):545–550

    PubMed  Google Scholar 

  • Levy JJ, Gassmuller J, Audring H, Brenke A, Albrecht-Nebe H (1993) [imaging subcutaneous atrophy in circumscribed scleroderma with 20 mhz b-scan ultrasound]. Hautarzt 44(7):446–451

    PubMed  CAS  Google Scholar 

  • Liu P, Uziel Y, Chuang S, Silverman E, Krafchik B, Laxer R (1994) Localized scleroderma: Imaging features. Pediatr Radiol 24:207–209

    PubMed  CAS  Google Scholar 

  • Luer W, Jockel D, Henze T, Schipper HI (1990) Progressive inflammatory lesions of the brain parenchyma in localized scleroderma of the head. J Neurol 237(6):379–381

    PubMed  CAS  Google Scholar 

  • Man J, Dytoc MT (2004) Use of imiquimod cream 5% in the treatment of localized morphea. J Cutan Med Surg 8(3):166–169

    PubMed  Google Scholar 

  • Mancuso G, Berdondini RM (2003) Topical tacrolimus in the treatment of localized scleroderma. Eur J Dermatol 13(6):590–592

    PubMed  Google Scholar 

  • Martini G, Murray KJ, Howell KJ, Harper J, Atherton D, Woo P, Zulian F, Black CM (2002) Juvenile-onset localized scleroderma activity detection by infrared thermography. Rheumatology (Oxford) 41(10):1178–1182

    CAS  Google Scholar 

  • Martini G, Ramanan AV, Falcini F, Girschick H, Goldsmith DP, Zulian F (2009) Successful treatment of severe or methotrexate-resistant juvenile localized scleroderma with mycophenolate mofetil. Rheumatology (Oxford, England) 48(11):1410–1413

    Google Scholar 

  • Maruotti N, Cantatore FP (2010) Vitamin d and the immune system. J Rheumatol 37(3):491–495

    PubMed  CAS  Google Scholar 

  • Marzano AV, Menni S, Parodi A, Borghi A, Fuligni A, Fabbri P, Caputo R (2003) Localized scleroderma in adults and children. Clinical and laboratory investigations on 239 cases. Eur J Dermatol 13(2):171–176

    PubMed  Google Scholar 

  • Mattila L, Airola K, Ahonen M, Hietarinta M, Black C, Saarialho-Kere U, Kahari VM (1998) Activation of tissue inhibitor of metalloproteinases-3 (timp-3) mrna expression in scleroderma skin fibroblasts. J Invest Dermatol 110(4):416–421

    PubMed  CAS  Google Scholar 

  • Mattozzi C, Richetta AG, Cantisani C, Giancristoforo S, D’Epiro S, Gonzalez Serva A, Viola F, Cucchiara S, Calvieri S (2010) Morphea, an unusual side effect of anti-tnf-alpha treatment. Eur J Dermatol 20(3):400–401

    PubMed  Google Scholar 

  • McColl G, Buchanan RR (1994) Unilateral scleroderma following ischemic hand injury [letter]. J Rheumatol 21(2):380–381

    PubMed  CAS  Google Scholar 

  • McNiff JM, Glusac EJ, Lazova RZ, Carroll CB (1999) Morphea limited to the superficial reticular dermis: An underrecognized histologic phenomenon. Am J Dermatopathol 21(4):315–319

    PubMed  CAS  Google Scholar 

  • Menni S, Marzano AV, Passoni E (1997) Neurologic abnormalities in two patients with facial hemiatrophy and sclerosis coexisting with morphea. Pediatr Dermatol 14(2): 113–116

    PubMed  CAS  Google Scholar 

  • Micalizzi C, Parodi A, Rebora A (1994) Morphea with nodular lesions. Br J Dermatol 131:298–300

    PubMed  CAS  Google Scholar 

  • Micalizzi C, Parodi A, Rebora A (1996) Generalized bullous morphoea. Efficacy of salazopyrin. Clin Exp Dermatol 21(3):246–247

    PubMed  CAS  Google Scholar 

  • Milano A, Pendergrass SA, Sargent JL, George LK, McCalmont TH, Connolly MK, Whitfield ML (2008) Molecular subsets in the gene expression signatures of scleroderma skin. PLoS One 3(7):e2696

    PubMed  Google Scholar 

  • Miller J (1992a) The fasciitis-morphea complex in children. Am J Dis Child 146(6):733–736

    PubMed  Google Scholar 

  • Miller JJ, 3rd (1992b) The fasciitis-morphea complex in children. American Journal of Diseases of Children (1960) 146(6):733–736

    Google Scholar 

  • Mitchet C, Doyle J, Ginsburg W (1981) Eosinophilic fascitis: Report of 15 cases. Mayo Clin Proc 56:27–34

    Google Scholar 

  • Mizutani H, Yoshida T, Nouchi N, Hamanaka H, Shimizu M (1999) Topical tocoretinate improved hypertrophic scar, skin sclerosis in systemic sclerosis and morphea. J Dermatol 26(1):11–17

    PubMed  CAS  Google Scholar 

  • Mohrenschlager M, Jung C, Ring J, Abeck D (1999) Effect of penicillin g on corium thickness in linear morphea of childhood: An analysis using ultrasound technique. Pediatr Dermatol 16(4):314–316

    PubMed  CAS  Google Scholar 

  • Moller R, Serup J, Ammitzboll T (1985) Glycosaminoglycans in localized scleroderma (morphoea). Connect Tissue Res 13(3):227–236

    PubMed  CAS  Google Scholar 

  • Monteiro MR, Murphy EE, Galaria NA, Whitaker-Menezes D, Murphy GF (2000) Cytological alterations in dermal dendrocytes in vitro: Evidence for transformation to a non-dendritic phenotype. Br J Dermatol 143(1):84–90

    PubMed  CAS  Google Scholar 

  • Morison WL (1997) Psoralen uva therapy for linear and generalized morphea. J Am Acad Dermatol 37(4):657–659

    PubMed  CAS  Google Scholar 

  • Morita A, Werfel T, Stege H, Ahrens C, Karmann K, Grewe M, Grether-Beck S, Ruzicka T, Kapp A, Klotz LO, Sies H, Krutmann J (1997) Evidence that singlet oxygen-induced human t helper cell apoptosis is the basic mechanism of ultraviolet-a radiation phototherapy. J Exp Med 186(10):1763–1768

    PubMed  CAS  Google Scholar 

  • Mork NJ (1981) Clinical and histopathologic morphea with immunological evidence of lupus erythematosus: A case report. Acta Derm Venereol 61(4):367–368

    PubMed  CAS  Google Scholar 

  • Morley SM, Gaylarde PM, Sarkany I (1985) Epidermal thickness in systemic sclerosis and morphoea. Clin Exp Dermatol 10(1):51–57

    PubMed  CAS  Google Scholar 

  • Moynahan EJ (1973) Morphoea (localized cutaneous scleroderma) treated with low-dosage penicillamine (4 cases, including coup de sabre). Proc R Soc Med 66(11): 1083–1085

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  • Narvaez D, Kanitakis J, Faure M, Claudy A (1996) Immunohistochemical study of cd34-positive dendritic cells of human dermis. Am J Dermatopathol 18(3):283–288

    PubMed  CAS  Google Scholar 

  • Neldner KH (1978) Treatment of localized linear scleroderma with phenytoin. Cutis 22(5):569–572

    PubMed  CAS  Google Scholar 

  • Neuhofer J, Fritsch P (1984) Treatment of localized scleroderma and lichen sclerosus with etretinate. Acta Derm Venereol 64(2):171–174

    PubMed  CAS  Google Scholar 

  • Neustadter JH, Samarin F, Carlson KR, Girardi M (dy2009) Extracorporeal photochemotherapy for generalized deep morphea. Arch Dermatol 145(2): 127–130

    PubMed  Google Scholar 

  • O’Leary P, Montgomery H, Ragsdale W (1957) Dermatohistopathology of various types of scleroderma. Arch Dermatol 75:78–87

    Google Scholar 

  • Oikarinen A, Knuutinen A (2001) Ultraviolet a sunbed used for the treatment of scleroderma. Acta Derm Venereol 81(6):432–433

    PubMed  CAS  Google Scholar 

  • Oyama N, Chan I, Neill SM, Hamada T, South AP, Wessagowit V, Wojnarowska F, D’Cruz D, Hughes GJ, Black MM, McGrath JA (2003) Autoantibodies to extracellular matrix protein 1 in lichen sclerosus. Lancet 362(9378):118–123

    PubMed  CAS  Google Scholar 

  • Oyama N, Chan I, Neill SM, South AP, Wojnarowska F, Kawakami Y, D’Cruz D, Mepani K, Hughes GJ, Bhogal BS, Kaneko F, Black MM, McGrath JA (2004) Development of antigen-specific elisa for circulating autoantibodies to extracellular matrix protein 1 in lichen sclerosus. J Clin Invest 113(11):1550–1559

    PubMed  CAS  Google Scholar 

  • Passos CO, Werneck CC, Onofre GR, Pagani EA, Filgueira AL, Silva LC (2003) Comparative biochemistry of human skin: Glycosaminoglycans from different body sites in normal subjects and in patients with localized scleroderma. J Eur Acad Dermatol Venereol 17(1):14–19

    PubMed  CAS  Google Scholar 

  • Perlish JS, Lemlich G, Fleischmajer R (1988) Identification of collagen fibrils in scleroderma skin. J Invest Dermatol 90(1):48–54

    PubMed  CAS  Google Scholar 

  • Person JR, Su WP (1979) Subcutaneous morphoea: A clinical study of sixteen cases. Br J Dermatol 100(4):371–380

    PubMed  CAS  Google Scholar 

  • Peter RU, Ruzicka T, Eckert F (1991) Low-dose cyclosporine a in the treatment of disabling morphea. Arch Dermatol 127(9):1420–1421

    PubMed  CAS  Google Scholar 

  • Peterson LS, Nelson AM, Su WP (1995) Classification of morphea (localized scleroderma). Mayo Clin Proc 70(11):1068–1076

    PubMed  CAS  Google Scholar 

  • 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

    PubMed  CAS  Google Scholar 

  • Rai R, Handa S, Gupta S, Kumar B (2000) Bilateral en coup de sabre-a rare entity. Pediatr Dermatol 17(3):222–224

    PubMed  CAS  Google Scholar 

  • Rencic A, Brinster N, Nousari CH (2003) Keloid morphea and nodular scleroderma: Two distinct clinical variants of scleroderma? J Cutan Med Surg 7(1):20–24

    PubMed  Google Scholar 

  • Rigby WF, Denome S, Fanger MW (1987) Regulation of lymphokine production and human t lymphocyte activation by 1,25-dihydroxyvitamin d3. Specific inhibition at the level of messenger rna. J Clin Invest 79(6): 1659–1664

    PubMed  CAS  Google Scholar 

  • Rigby WF, Waugh MG (1992) Decreased accessory cell function and costimulatory activity by 1,25-dihydroxyvitamin d3-treated monocytes. Arthritis Rheum 35(1):110–119

    PubMed  CAS  Google Scholar 

  • Rodnan GP, Lipinski E, Luksick J (1979) Skin thickness and collagen content in progressive systemic sclerosis and localized scleroderma. Arthritis Rheum 22(2): 130–140

    PubMed  CAS  Google Scholar 

  • Rose CD, Fawcett PT, Gibney K, Doughty RA, Singsen BH (1994) Serial measurements of soluble interleukin 2 receptor levels (sil2-r) in children with juvenile rheumatoid arthritis treated with oral methotrexate. Ann Rheum Dis 53(7):471–474

    PubMed  CAS  Google Scholar 

  • Rosenberg AM, Uziel Y, Krafchik BR, Hauta SA, Prokopchuk PA, Silverman ED, Laxer RM (1995) Antinuclear antibodies in children with localized scleroderma. J Rheumatol 22(12):2337–2343

    PubMed  CAS  Google Scholar 

  • Ruffatti A, Peserico A, Rondinone R, Calligaro A, Del Ross T, Ghirardello A, Germino M, Todesco S (1991) Prevalence and characteristics of anti-single-stranded DNA antibodies in localized scleroderma. Comparison with systemic lupus erythematosus. Arch Dermatol 127(8): 1180–1183

    PubMed  CAS  Google Scholar 

  • Sakuraoka K, Tajima S, Nishikawa T (1992) Progressive facial hemiatrophy: Report of five cases and biochemical analysis of connective tissue. Dermatology 185(3):196–201

    PubMed  CAS  Google Scholar 

  • Salmon-Ehr V, Serpier H, Nawrocki B, Gillery P, Clavel C, Kalis B, Birembaut P, Maquart FX (1996) Expression of interleukin-4 in scleroderma skin specimens and scleroderma fibroblast cultures. Potential role in fibrosis. Arch Dermatol 132(7):802–806

    PubMed  CAS  Google Scholar 

  • Sato S, Fujimoto M, Hasegawa M, Takehara K (2003) Antiphospholipid antibody in localised scleroderma. Ann Rheum Dis 62(8):771–774

    PubMed  CAS  Google Scholar 

  • Sato S, Fujimoto M, Ihn H, Kikuchi K, Takehara K (1994) Clinical characteristics associated with antihistone antibodies in patients with localized scleroderma. J Am Acad Dermatol 31(4):567–571

    PubMed  CAS  Google Scholar 

  • Sato S, Fujimoto M, Kikuchi K, Ihn H, Tamaki K, Takehara K (1996a) Elevated soluble cd23 levels in the sera from patients with localized scleroderma. Arch Dermatol Res 288(2):74–78

    PubMed  CAS  Google Scholar 

  • Sato S, Fujimoto M, Kikuchi K, Ihn H, Tamaki K, Takehara K (1996b) Soluble cd4 and cd8 in serum from patients with localized scleroderma. Arch Dermatol Res 288(7):358–362

    PubMed  CAS  Google Scholar 

  • Sator PG, Radakovic S, Schulmeister K, Honigsmann H, Tanew A (2009) Medium-dose is more effective than low-dose ultraviolet a1 phototherapy for localized scleroderma as shown by 20-mhz ultrasound assessment. J Am Acad Dermatol 60(5):786–791

    PubMed  Google Scholar 

  • Schaffer JV, Carroll C, Dvoretsky I, Huether MJ, Girardi M (2000) Postirradiation morphea of the breast presentation of two cases and review of the literature. Dermatology 200(1):67–71

    PubMed  CAS  Google Scholar 

  • Scharffetter K, Lankat-Buttgereit B, Krieg T (1988) Localization of collagen mrna in normal and scleroderma skin by in-situ hybridization. Eur J Clin Invest 18(1):9–17

    PubMed  CAS  Google Scholar 

  • Scharffetter-Kochanek K, Goldermann R, Lehmann P, Holzle E, Goerz G (1995) Puva therapy in disabling pansclerotic morphoea of children [letter]. Br J Dermatol 132(5):830–831

    PubMed  CAS  Google Scholar 

  • Schiener R, Behrens-Williams SC, Gottlober P, Pillekamp H, Peter RU, Kerscher M (2000) Eosinophilic fasciitis treated with psoralen-ultraviolet a bath photochemotherapy. Br J Dermatol 142(4):804–807

    PubMed  CAS  Google Scholar 

  • Schlaak M, Friedlein H, Kauer F, Renner R, Rogalski C, Simon JC (2008) Successful therapy of a patient with therapy recalcitrant generalized bullous scleroderma by extracorporeal photopheresis and mycophenolate mofetil. J Eur Acad Dermatol Venerol 22(5):631–633

    CAS  Google Scholar 

  • Seitz M, Loetscher P, Dewald B, Towbin H, Rordorf C, Gallati H, Baggiolini M, Gerber NJ (1995) Methotrexate action in rheumatoid arthritis: Stimulation of cytokine inhibitor and inhibition of chemokine production by peripheral blood mononuclear cells. Br J Rheumatol 34(7):602–609

    PubMed  CAS  Google Scholar 

  • Seitz M, Zwicker M, Loetscher P (1998) Effects of methotrexate on differentiation of monocytes and production of cytokine inhibitors by monocytes. Arthritis Rheum 41(11):2032–2038

    PubMed  CAS  Google Scholar 

  • Seyger MM, van den Hoogen FH, de Boo T, de Jong EM (1997) Reliability of two methods to assess morphea: Skin scoring and the use of a durometer. J Am Acad Dermatol 37 (5 Pt 1):793–796

    PubMed  CAS  Google Scholar 

  • Seyger MM, van den Hoogen FH, de Boo T, de Jong EM (1998) Low-dose methotrexate in the treatment of widespread morphea. J Am Acad Dermatol 39 (2 Pt 1):220–225

    PubMed  CAS  Google Scholar 

  • Seyger MM, van den Hoogen FH, van Vlijmen-Willems IM, van de Kerkhof PC, de Jong EM (2001) Localized and systemic scleroderma show different histological responses to methotrexate therapy. J Pathol 193(4):511–516

    PubMed  CAS  Google Scholar 

  • Sgonc R, Gruschwitz MS, Dietrich H, Recheis H, Gershwin ME, Wick G (1996) Endothelial cell apoptosis is a primary pathogenetic event underlying skin lesions in avian and human scleroderma. J Clin Invest 98(3):785–792

    PubMed  CAS  Google Scholar 

  • Shulman L (1974) Diffuse fasciitis with hypergammaglobulinemia and eosinophilia: A new syndrome? Clinical Reaserch 23:443A

    Google Scholar 

  • Silman A, Jannini S, Symmons D, Bacon P (1988) An epidemiological study of scleroderma in the west midlands. Br J Rheumatol 27(4):286–290

    PubMed  CAS  Google Scholar 

  • Skobieranda K, Helm KF (1995) Decreased expression of the human progenitor cell antigen (cd34) in morphea. Am J Dermatopathol 17(5):471–475

    PubMed  CAS  Google Scholar 

  • Soma Y, Fujimoto M (1998) Frontoparietal scleroderma (en coup de sabre) following blaschko’s lines. J Am Acad Dermatol 38 (2 Pt 2):366–368

    PubMed  CAS  Google Scholar 

  • Soma Y, Tamaki T, Kikuchi K, Abe M, Igarashi A, Takehara K, Ishibashi Y (1993) Coexistence of morphea and systemic sclerosis. Dermatology 186:103–105

    PubMed  CAS  Google Scholar 

  • Stava Z, Kobikova M (1977) Salazopyrin in the treatment of scleroderma. Br J Dermatol 96(5):541–544

    PubMed  CAS  Google Scholar 

  • Stefanaki C, Stefanaki K, Kontochristopoulos G, Antoniou C, Stratigos A, Nicolaidou E, Gregoriou S, Katsambas A (2008) Topical tacrolimus 0.1% ointment in the treatment of localized scleroderma. An open label clinical and histological study. J Dermatol 35(11):712–718

    PubMed  Google Scholar 

  • Stefanec T (2000) Endothelial apoptosis: Could it have a role in the pathogenesis and treatment of disease? Chest 117(3):841–854

    PubMed  CAS  Google Scholar 

  • Stege H, Berneburg M, Humke S, Klammer M, Grewe M, Grether-Beck S, Boedeker R, Diepgen T, Dierks K, Goerz G, Ruzicka T, Krutmann J (1997) High-dose uva1 radiation therapy for localized scleroderma [see comments]. J Am Acad Dermatol 36 (6 Pt 1):938–944

    PubMed  CAS  Google Scholar 

  • Steger JW, Matthews JH (1999) Uva therapy for scleroderma. Journal of the American Academy of Dermatology 40 (5 Pt 1):787–788

    PubMed  CAS  Google Scholar 

  • Stone J, Franks AJ, Guthrie JA, Johnson MH (2001) Scleroderma «En coup de sabre»: Pathological evidence of intracerebral inflammation. J Neurol Neurosurg Psychiatry 70(3):382–385

    PubMed  CAS  Google Scholar 

  • Strauss RM, Bhushan M, Goodfield MJ (2004) Good response of linear scleroderma in a child to ciclosporin. The British journal of dermatology 150(4):790–792

    PubMed  CAS  Google Scholar 

  • Su WP, Greene SL (1986) Bullous morphea profunda. Am J Dermatopathol 8(2):144–147

    PubMed  CAS  Google Scholar 

  • Su WP, Person JR (1981) Morphea profunda. A new concept and a histopathologic study of 23 cases. Am J Dermatopathol 3(3):251–260

    PubMed  CAS  Google Scholar 

  • Takehara K, Moroi Y, Nakabayashi Y, Ishibashi Y (1983) Antinuclear antibodies in localized scleroderma. Arthritis Rheum 26(5):612–616

    PubMed  CAS  Google Scholar 

  • Taveira M, Selores M, Costa V, Massa A (1999) Generalized morphea and lichen sclerosus et atrophicus successfully treated with sulphasalazine [letter]. J Eur Acad Dermatol Venereol 12(3):283–284

    PubMed  CAS  Google Scholar 

  • Tay YK (2003) Topical calcipotriol ointment in the treatment of morphea. J Dermatolog Treat 14(4):219–221

    PubMed  CAS  Google Scholar 

  • Todd DJ, Askari A, Ektaish E (1998) Puva therapy for disabling pansclerotic morphoea of children [letter]. Br J Dermatol 138(1):201–202

    PubMed  CAS  Google Scholar 

  • Tollefson MM, Witman PM (2007) En coup de sabre morphea and parry-romberg syndrome: A retrospective review of 54 patients. Journal of the American Academy of Dermatology 56(2):257–263

    PubMed  Google Scholar 

  • Torres JE, Sanchez JL (1998) Histopathologic differentiation between localized and systemic scleroderma. Am J Dermatopathol 20(3):242–245

    PubMed  CAS  Google Scholar 

  • Tuffanelli DL (1998) Localized scleroderma. Semin Cutan Med Surg 17(1):27–33

    PubMed  CAS  Google Scholar 

  • Uitto J, Santa Cruz DJ, Bauer EA, Eisen AZ (1980) Morphea and lichen sclerosus et atrophicus. Clinical and histopathologic studies in patients with combined features. J Am Acad Dermatol 3(3):271–279

    PubMed  CAS  Google Scholar 

  • Umbert P, Winkelmann RK (1978) Concurrent localized scleroderma and discoid lupus erythematosus. Cutaneous ‘mixed’ or ‘overlap’ syndrome. Arch Dermatol 114(10):1473–1478

    PubMed  CAS  Google Scholar 

  • Unterberger I, Trinka E, Engelhardt K, Muigg A, Eller P, Wagner M, Sepp N, Bauer G (2003) Linear scleroderma “En coup de sabre” Coexisting with plaque-morphea: Neuroradiological manifestation and response to corticosteroids. J Neurol Neurosurg Psychiatry 74(5):661–664

    PubMed  CAS  Google Scholar 

  • Uziel Y, Feldman BM, Krafchik BR, Yeung RS, Laxer RM (2000) Methotrexate and corticosteroid therapy for pediatric localized scleroderma. J Pediatr 136(1):91–95

    PubMed  CAS  Google Scholar 

  • Uziel Y, Krafchik BR, Feldman B, Silverman ED, Rubin LA, Laxer RM (1994a) Serum levels of soluble interleukin-2 receptor. A marker of disease activity in localized scleroderma. Arthritis Rheum 37(6):898–901

    PubMed  CAS  Google Scholar 

  • Uziel Y, Krafchik BR, Silverman ED, Thorner PS, Laxer RM (1994b) Localized scleroderma in childhood: A report of 30 cases. Semin Arthritis Rheum 23(5):328–340

    PubMed  CAS  Google Scholar 

  • van Bergen BH, van Dooren-Greebe RJ, Fiselier TJ, Koopman RJ (1997) [d-penicillamine in treatment of scleroderma “En coup de sabre”]. Hautarzt 48(1):42–44

    PubMed  Google Scholar 

  • van den Hoogen FH, Boerbooms AM, Swaak AJ, Rasker JJ, van Lier HJ, van de Putte LB (1996) Comparison of methotrexate with placebo in the treatment of systemic sclerosis: A 24 week randomized double-blind trial, followed by a 24 week observational trial. Br J Rheumatol 35(4):364–372

    PubMed  Google Scholar 

  • Venneker GT, Das PK, Naafs B, Tigges AJ, Bos JD, Asghar SS (1994) Morphoea lesions are associated with aberrant expression of membrane cofactor protein and decay accelerating factor in vascular endothelium. Br J Dermatol 131(2):237–242

    PubMed  CAS  Google Scholar 

  • Wach F, Ullrich H, Schmitz G, Landthaler M, Hein R (1995) Treatment of severe localized scleroderma by plasmapheresis — report of three cases. Br J Dermatol 133(4):605–609

    PubMed  CAS  Google Scholar 

  • Wakelin SH, James MP (1995) Zosteriform atrophoderma of pasini and pierini. Clin Exp Dermatol 20(3):244–246

    PubMed  CAS  Google Scholar 

  • Walters R, Pulitzer M, Kamino H (2009) Elastic fiber pattern in scleroderma / morphea. J Cutan Pathol 36(9):952–957

    PubMed  Google Scholar 

  • Weedon D (1997) Disorders of collagen. In: Skin pathology. 1st edn. Churchill Livingstone, Hong-Kong, pp 288–293

    Google Scholar 

  • Weibel L, Sampaio MC, Visentin MT, Howell KJ, Woo P, Harper JI (2006) Evaluation of methotrexate and corticosteroids for the treatment of localized scleroderma (morphoea) in children. The British journal of dermatology 155(5):1013–1020

    PubMed  CAS  Google Scholar 

  • Weide B, Walz T, Garbe C (2000) Is morphoea caused by borrelia burgdorferi? A review. Br J Dermatol 142(4):636–644

    PubMed  CAS  Google Scholar 

  • Whittaker SJ, Smith NP, Jones RR (1989) Solitary morphoea profunda. Br J Dermatol 120(3):431–440

    PubMed  CAS  Google Scholar 

  • Winkelmann R (1985) Localized cutaneous scleroderma. Seminars in Dermatology 4(2):90–103

    Google Scholar 

  • Winkelmann RK, Connolly SM, Doyle JA (1982) Carpal tunnel syndrome in cutaneous connective tissue disease: Generalized morphea, lichen sclerosus, fasciitis, discoid lupus erythematosus, and lupus panniculitis. J Am Acad Dermatol 7(1):94–99

    PubMed  CAS  Google Scholar 

  • Wlaschek M, Briviba K, Stricklin GP, Sies H, Scharffetter-Kochanek K (1995) Singlet oxygen may mediate the ultraviolet a-induced synthesis of interstitial collagenase. J Invest Dermatol 104(2):194–198

    PubMed  CAS  Google Scholar 

  • Wollenberg A, Baumann L, Plewig G (1995) Linear atrophoderma of moulin: A disease which follows blaschko’s lines. Br J Dermatol 135:277–279

    Google Scholar 

  • Wollina U, Looks A, Schneider R, Maak B (1998) Disabling morphoea of childhood-beneficial effect of intravenous immunoglobulin therapy [letter]. Clin Exp Dermatol 23(6):292–293

    PubMed  CAS  Google Scholar 

  • Wuthrich RC, Roenigk HH, Steck WD (1975) Localized scleroderma. Arch Dermatol 111(1):98–100

    PubMed  CAS  Google Scholar 

  • Yamaguchi K, Takeuchi I, Yoshii N, Gushi A, Kanekura T, Kanzaki T (1998) The discrepancy in hardness between clinical and histopathological findings in localized scleroderma treated with puva. J Dermatol 25(8):544–546

    PubMed  CAS  Google Scholar 

  • Yamanaka CT, Gibbs NF (1999) Trauma-induced linear scleroderma. Cutis 63(1):29–32

    PubMed  CAS  Google Scholar 

  • Yamane K, Ihn H, Kubo M, Yazawa N, Kikuchi K, Soma Y, Tamaki K (2000) Increased serum levels of soluble vascular cell adhesion molecule 1 and e-selectin in patients with localized scleroderma. J Am Acad Dermatol 42 (1 Pt 1):64–69

    PubMed  CAS  Google Scholar 

  • Young EM, Jr., Barr RJ (1985) Sclerosing dermatoses. J Cutan Pathol 12(5):426–441

    PubMed  Google Scholar 

  • Zheng XY, Zhang JZ, Tu P, Ma SQ (1998) Expression of platelet-derived growth factor b-chain and platelet-derived growth factor beta-receptor in fibroblasts of scleroderma. J Dermatol Sci 18(2):90–97

    PubMed  CAS  Google Scholar 

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Kellet, C.V., Orteu, C.H., Dutz, J.P. (2011). Localized Scleroderma. In: Hertl, M. (eds) Autoimmune Diseases of the Skin. Springer, Vienna. https://doi.org/10.1007/978-3-211-99225-8_7

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