Current concepts of scleroderma

Gurskiĭ G.É.; Grebennikov V.A.; Khoron'ko V.V.

doi:https://doi.org/

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Abstract:

The present review considers the data extracted from various literature sources concerning peculiarities of the clinical picture of circumscribed scleroderma. The authors discuss the hypothesis that regards circumscribed and systemic scleroderma as manifestations of one and the same pathological process. This view is based on the reports of unidirectional metabolic disturbances, common mechanisms underlying the development of pathohistological changes of the skin in both forms of the disease, and transformation of the localized process into progressive systemic sclerosis. The data on the incidence and predominance of different forms of circumscribed scleroderma are presented in conjunction with the information about their clinical symptoms, duration of the disease and its relation to other pathological conditions. Different therapeutic modalities (both multi-course and combined) for the treatment of circumscribed scleroderma are analysed.

Keywords:

circumscribed scleroderma

antibiotics

chemotherapy

methotrexate

corticosteroids

photochemotherapy

Authors:

Gurskiĭ G.É.

GBOU VPO "Rostovskiĭ gosudarstvennyĭ meditsinskiĭ universitet", Rostov-na-Donu

Grebennikov V.A.

Rostov State Medical University, Rostov-on-Don

Khoron'ko V.V.

GBOU VPO "Rostovskiĭ gosudarstvennyĭ meditsinskiĭ universitet", Rostov-na-Donu

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References:

Guseva N.G. Sistemnaya sklerodermiya: klinika, diagnostika, lechenie. Ros zhurn kozh i ven bol 2002; 4: 5-15.
Connolly M.K. Pulmonary Arterial Hypertension in Scleroderma: A New Treatment. Scleroderma Voice 2002; 2. www.scleroderma.org/medical/pulmonary_articles/ Connolly 2002. shtm: 32k.
Sjogren R.W. Gastrointestinal features of scleroderma. Curr Opin Rheumatol 1996; 8: 6: 569-575.
Pathak R., Gabor A.J. Scleroderma and central nervous system vasculitis. Stroke 1991; 22: 3: 410-413.
Gallyamova Yu.A. Ochagovaya sklerodermiya. Lech vrach 2008; 5.
Zulian F. Systemic manifestations in localized scleroderma. Curr Rheumatol Rep 2004; 6: 6: 417-424.
Bolotnaya L.A., Serbina I.M. Sovremennaya patogeneticheskaya terapiya sklerodermii. Mezhdunarodnyi med zhurn 1999; 3: 56-58.
Korobeinikova E.A., Martynova L.M., Anisimova A.V. Klinicheskie aspekty ogranichennoi sklerodermii. Ros zhurn kozhn i ven bol 2004; 3: 27-29.
Furst A. Scleroderma: a fascinating, troubling disease. Adv Practice Nursing J 2004; 4: 2.
Bolotnaya L.A., Shakhova F.B., Serbina I.M. Novoe v patogeneze i terapii ogranichennoi sklerodermii. Vestn dermatol i venerol 2004; 2: 31-34.
Alexandrescu D.T., Bhagwati N.S., Wiernik P.H. Chemotherapy-induced scleroderma: a pleiomorphic syndrome. Clin Exp Dermatol 2005; 30: 2: 141-145.
Gliddon A.E., Doré C.J., Black C.M. et al. Prevention of vascular damage in scleroderma and autoimmune Raynaud's phenomenon: a multicenter, randomized, double-blind, placebo-controlled trial of the angiotensin-converting enzyme inhibitor quinapril. Arthritis Rheum 2007; 56: 11: 3837-3846.
Dovzhanskii S.I. Kliniko-immunologicheskie paralleli pri ogranichennoi i sistemnoi sklerodermii. Ros zhurn kozhn i ven bol 2002; 4: 26-29.
Asano Y., Ihn H., Jinnin M. et al. Involvement of alphavbeta5 integrin in the establishment of autocrine TGF-beta signaling in dermal fibroblasts derived from localized scleroderma. J Invest Dermatol 2006; 126: 8: 1761-1769.
Milenkovic S., Petrovic L., Risimic D. et al. Choroidal sclerosis in localized Scleroderma (morphea en plaque). Ophth Res 2008; 40: 2: 101-104.
Carlson J.A., Chen K.R. Cutaneous vasculitis update: neutrophilic muscular vessel and eosinophilic, granulomatous and lymphocytic vasculitis syndromes. Am J Dermatopathol 2007; 29: 1: 32-43.
Potekaev N.S., Potekaev N.N. Bolezn' Laima i obuslovlennye eyu porazheniya kozhi. Vestn dermatol i venerol 2006; 6: 3-9.
Bonifati C., Impara G., Morrone A. et al. Simultaneous occurrence of linear scleroderma and homolateral segmental vitiligo. J Eur Acad Dermatol Venereol 2006; 20: 1: 63-65.
Christen-Zaech S., Hakim M.D., Afsar F.S., Paller A.S. Pediatric morphea (localized scleroderma): review of 136 patients. J Am Acad Dermatol 2008; 59: 3: 385-396.
Dehen L., Roujeau J.C., Cosnes A., Revuz J. Internal involvement in localized scleroderma. Medicine (Baltimore) 1994; 73: 5: 241-245.
González-López M.A., Drake M., González-Vela M.C. et al. Generalized morphea and primary biliary cirrhosis coexisting in a male patient. J Dermatol 2006; 33: 10: 709-713.
Göring H.D., Panzner M., Lakotta W., Ziemer A. Coincidence of scleroderma and primary biliary cirrhosis. Results of a systematic study of a dermatologic patient sample. Hautarzt 1998; 49: 5: 361-366.
Hayakawa I., Hasegawa M., Takehara K., Sato S. Anti-DNA topoisomerase IIalpha autoantibodies in localized scleroderma. Arthr Rheum 2004; 50: 1: 227-232.
Zulian F., Vallongo C., Woo P. et al. Localized scleroderma in childhood is not just a skin disease. Arthrs Rheum 2005; 52: 9: 2872-2881.
Laxer R.M., Feldman B.M. General and local scleroderma in children and dermatomyositis and associated syndromes. Curr Opin Rheumatol 1997; 9: 5: 458-464.
Laxer R.M., Zulian F. Localized scleroderma. Curr Opin Rheumatol 2006; 18: 6: 606-613.
Peterson L.S., Nelson A.M., Su W.P. Classification of morphea (localized scleroderma). Mayo Clin Proc 1995; 70: 11: 1068-1076.
Bolotnaya L.A., Serbina I.M. Sovremennaya patogeneticheskaya terapiya sklerodermii. Mezhdunarodnyi med zhurn 1999; 3: 56-58.
Smirnov A.V., Kurnikov G.Yu., Perestoronina V.S. KVCh-terapiya v lechenii ogranichennoi sklerodermii. Millimetrovye volny v biologii i meditsine 2000; 2: 18: 34-35.
Kokolina V.F., Kharybina E.I., Ukolova I.L., Kireeva E.S. Kliniko-immunologicheskie osobennosti i modifikatsiya kompleksnogo lecheniya pri ochagovoi sklerodermii naruzhnykh genitalii u devochek s pomoshch'yu kursovoi nizkointensivnoi lazernoi terapii. Pediatriya 2001; 3: 106-108.
Gruss C.J., Von Kobyletzki G., Behrens-Williams S.C. et al. Effects of low dose ultraviolet A-1 phototherapy on morphea. Photodermatol Photoimmunol Photomed 2001; 17: 4: 149-155.
Kreuter A., Gambichler T., Avermaete A. et al. Combined treatment with calcipotriol ointment and low-dose ultraviolet A1 phototherapy in childhood morphea. Pediatr Dermatol 2001; 18: 3: 241-245.
Gliddon A.E., Doré C.J., Black C.M. et al. Prevention of vascular damage in scleroderma and autoimmune Raynaud's phenomenon: a multicenter, randomized, double-blind, placebo-controlled trial of the angiotensin-converting enzyme inhibitor quinapril. Arthr Rheum 2007; 56: 11: 3837-3846.
Cassidy J.T., Petty R.E. The sclerodermas and related disease. Textbook of Pediatrics Rheumatology. 4th ed. Pheladelphia 2001; 505-541.
Unterberger I., Trinka E., Engelhardt K. et al. Linear scleroderma "en coup de sabre" coexisting with plaque-morphea: neuroradiological manifestation and response to corticosteroids. J Neurol Neurosurg Psychiat 2003; 74: 5: 661-664.
Kreuter A., Gambichler T., Breuckmann F. et al. Pulsed high-dose corticosteroids combined with low-dose methotrexate in severe localized scleroderma. Arch Dermatol 2005; 141: 7: 847-852.
Falanga V., Medsger T.A. Jr. D-penicillamine in the treatment of localized scleroderma. Arch Dermatol 1990; 126: 5: 609-612.
Hilário M.O. Esclerodermia. Oliveira SK, Azevedo EC. Reumatologia Pediátrica. Rio de Janeiro: Revinter 2000; 295: 476-480.
Uziel Y., Feldman B.M., Krafchik B.R. et al. Methotrexate and corticosteroid therapy for pediatric localized scleroderma. J Pediatr 2000; 136: 1: 91-95.
Cunningham B.B., Landells I.D., Langman C. et al. Topical calcipotriene for morphea/linear scleroderma. J Am Acad Dermatol 1998; 39: 2: Pt 1: 211-215.
Eubanks L.E., McBurney E.I., Galen W., Reed R. Linear scleroderma in children. Int J Dermatol 1996; 35: 5: 330-336.
Kerscher M., Volkenandt M., Gruss C. et al. Low-dose UVA phototherapy for treatment of localized scleroderma. J Am Acad Dermatol 1998; 38: 1: 21-26.
Scharffetter-Kochanek K., Goldermann R., Lehmann P. et al. PUVA therapy in disabling pansclerotic morphea of children. Br J Dermatol 1995; 132: 5: 830-831.
Gruss C., Stucker M., Kobyletzki G. et al. Low dose UVA1 phototherapy in disabling pansclerotic morphoea of childhood. Br J Dermatol 1997; 136: 2: 293-294.
Gambichler T., Kreuter A., Rotterdam S. et al. Linear scleroderma "en coup de sabre" treated with topical calcipotriol and psoralen plus ultraviolet A. J Eur Acad Dermatol Venereol 2003; 17: 5: 601-602.
Kreuter A., Gambichler T., Avermaete A. et al. Combined treatment with calcipotriol ointment and low-dose ultraviolet A1 phototherapy in childhood morphea. Pediat Dermatol 2001; 18: 3: 241-245.
Wach F., Ullrich H., Schmitz G. et al. Treatment of severe localized scleroderma by plasmapheresis - report of three cases. Br J Dermatol 1995; 133: 4: 605-609.
Lam G.K., Hummers L.K., Woods A., Wigley F.M. Efficacy and Safety of Etanercept in the Treatment of Scleroderma-Associated Joint Disease. J. Reumatol 2007; 34: 7: 1636-1637.
Oliver S.J., Moreira A., Kaplan G. Immune stimulation in scleroderma patients treated with thalidomide. Clin Immunol 2000; 97: 2: 109-120.
Man J., Dytoc M.T. Use of Imiquimod cream 5% in the treatment of localized morphea. J Cutan Med Surg 2004; 8: 3: 166-169.