Top

Published in:

01-02-2012 | SCLERODERMA (J VARGA, SECTION EDITOR)

Scleroderma Mimics

Authors: Jennifer Nashel, Virginia Steen

Published in: Current Rheumatology Reports | Issue 1/2012

Abstract

Scleroderma is a rare systemic autoimmune disease with multiple organ manifestations, including skin fibrosis. The groups of disorders classified as scleroderma mimics share the common thread of skin thickening but are otherwise quite incongruous in terms of underlying disease process and other organ involvement. This article reviews the clinical presentation, etiology, and treatment options available for scleroderma mimics, including morphea, scleredema, diabetic cheiroarthropathy, scleromyxedema, nephrogenic systemic fibrosis, and eosinophilic fasciitis. Through greater understanding of these diseases and the associated extradermal implications, we hope to facilitate recognition of scleroderma and its mimics.

• Fett N, Werth VP. Update on morphea: part I. Epidemiology, clinical presentation, and pathogenesis. J Am Acad Dermatol 2011; 64(2):217–28. This is an excellent review of the spectrum of morphea and localized scleroderma.

Zulian F, Athreya BH, Laxer R, Nelson AM, Feitosa de Oliveira SK, Punaro MG et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford) 2005.

• Fett N, Werth VP. Update on morphea: part II. Outcome measures and treatment. J Am Acad Dermatol 2011; 64(2):231–42. This is an important summary on the direction in which outcome measures and treatment of localized scleroderma are moving.

• Wortsman X, Gutierrez M, Saavedra T, Honeyman J. The role of ultrasound in rheumatic skin and nail lesions: a multi-specialist approach. Clin Rheumatol 2011; 30(6):739–48. This is a discussion of the use of ultrasound in evaluation of scleroderma skin.

Kroft EB, Creemers MC, van den Hoogen FH, Boezeman JB, de Jong EM. 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. 2009;160(5):1075–82.PubMedCrossRef

• Zulian F, Martini G, Vallongo C, Vittadello F, Falcini F, Patrizi A et al. Methotrexate treatment in juvenile localized scleroderma: a randomized, double-blind, placebo-controlled trial. Arthritis Rheum 2011; 63(7):1998–2006. This is an excellent example of how a randomized controlled trial can be carried out in this rare disease.

Kreuter A, Hyun J, Stucker M, Sommer A, Altmeyer P, Gambichler T. 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. 2006;54(3):440–7.PubMedCrossRef

Kreuter A, Hyun J, Skrygan M, Sommer A, Tomi NS, Breuckmann F, et al. Ultraviolet A1 phototherapy decreases inhibitory SMAD7 gene expression in localized scleroderma. Arch Dermatol Res. 2006;298(6):265–72.PubMedCrossRef

Kreuter A, Hyun J, Skrygan M, Sommer A, Bastian A, Altmeyer P, et al. Ultraviolet A1-induced downregulation of human beta-defensins and interleukin-6 and interleukin-8 correlates with clinical improvement in localized scleroderma. Br J Dermatol. 2006;155(3):600–7.PubMedCrossRef

10.

Beers WH, Ince A, Moore TL. Scleredema adultorum of Buschke: a case report and review of the literature. Semin Arthritis Rheum. 2006;35(6):355–9.PubMedCrossRef

11.

Dziadzio M, Anastassiades CP, Hawkins PN, Potter M, Gabrielli A, Brough GM, et al. From scleredema to AL amyloidosis: disease progression or coincidence? Review of the literature. Clin Rheumatol. 2006;25(1):3–15.PubMedCrossRef

12.

Meguerditchian C, Jacquet P, Beliard S, Benderitter T, Valero R, Carsuzza F, et al. Scleredema adultorum of Buschke: an under recognized skin complication of diabetes. Diabetes Metab. 2006;32(5 Pt 1):481–4.PubMedCrossRef

13.

Ikeda Y, Suehiro T, Abe T, Yoshida T, Shinoki T, Tahara K, et al. Severe diabetic scleredema with extension to the extremities and effective treatment using prostaglandin E1. Intern Med. 1998;37(10):861–4.PubMedCrossRef

14.

Thumpimukvatana N, Wongpraparut C, Lim HW. Scleredema diabeticorum successfully treated with ultraviolet A1 phototherapy. J Dermatol. 2010;37(12):1036–9.PubMedCrossRef

15.

Alsaeedi SH, Lee P. Treatment of scleredema diabeticorum with tamoxifen. J Rheumatol. 2010;37(12):2636–7.PubMedCrossRef

16.

Rosenbloom AL. Limitation of finger joint mobility in diabetes mellitus. J Diabet Complications. 1989;3(2):77–87.PubMedCrossRef

17.

Rosenbloom AL. Diabetic thick skin and stiff joints. Diabetologia. 1989;32(1):74–6.PubMedCrossRef

18.

Blum M, Wigley FM, Hummers LK. Scleromyxedema: a case series highlighting long-term outcomes of treatment with intravenous immunoglobulin (IVIG). Medicine (Baltimore). 2008;87(1):10–20.CrossRef

19.

Pomann JJ, Rudner EJ. Scleromyxedema revisited. Int J Dermatol. 2003;42(1):31–5.PubMedCrossRef

20.

Rongioletti F, Rebora A. Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema. J Am Acad Dermatol. 2001;44(2):273–81.PubMedCrossRef

21.

Dinneen AM, Dicken CH. Scleromyxedema. J Am Acad Dermatol. 1995;33(1):37–43.PubMedCrossRef

22.

Donato ML, Feasel AM, Weber DM, Prieto VG, Giralt SA, Champlin RE, et al. Scleromyxedema: role of high-dose melphalan with autologous stem cell transplantation. Blood. 2006;107(2):463–6.PubMedCrossRef

23.

Laimer M, Namberger K, Massone C, Koller J, Emberger M, Pleyer L, et al. Vincristine, idarubicin, dexamethasone and thalidomide in scleromyxoedema. Acta Derm Venereol. 2009;89(6):631–5.PubMedCrossRef

24.

Cowper SE, Robin HS, Steinberg SM, Su LD, Gupta S, LeBoit PE. Scleromyxoedema-like cutaneous diseases in renal-dialysis patients. Lancet. 2000;356(9234):1000–1.PubMedCrossRef

25.

• Morcos SK, Haylor J. Pathophysiology of nephrogenic systemic fibrosis: a review of experimental data. World J Radiol 2010; 2(11):427–33. This is an important summary of the relationship of gadolinium to NSF.

26.

Perez-Rodriguez J, Lai S, Ehst BD, Fine DM, Bluemke DA. Nephrogenic systemic fibrosis: incidence, associations, and effect of risk factor assessment—report of 33 cases. Radiology. 2009;250(2):371–7.PubMedCrossRef

27.

Schieren G, Wirtz N, Altmeyer P, Rump LC, Weiner SM, Kreuter A. Nephrogenic systemic fibrosis—a rapidly progressive disabling disease with limited therapeutic options. J Am Acad Dermatol. 2009;61(5):868–74.PubMedCrossRef

28.

Marckmann P, Skov L. Nephrogenic systemic fibrosis: clinical picture and treatment. Radiol Clin North Am. 2009;47(5):833–40. vi.PubMedCrossRef

29.

Kreuter A, Gambichler T, Weiner SM, Schieren G. Limited effects of UV-A1 phototherapy in 3 patients with nephrogenic systemic fibrosis. Arch Dermatol. 2008;144(11):1527–9.PubMedCrossRef

30.

Kay J, High WA. Imatinib mesylate treatment of nephrogenic systemic fibrosis. Arthritis Rheum. 2008;58(8):2543–8.PubMedCrossRef

31.

Cuffy MC, Singh M, Formica R, Simmons E, Abu Alfa AK, Carlson K, et al. Renal transplantation for nephrogenic systemic fibrosis: a case report and review of the literature. Nephrol Dial Transplant. 2011;26(3):1099–101.PubMedCrossRef

32.

Shulman LE. Diffuse fasciitis with eosinophilia: a new syndrome? Trans Assoc Am Physicians. 1975;88:70–86.PubMed

33.

Antic M, Lautenschlager S, Itin PH. Eosinophilic fasciitis 30 years after—what do we really know? Report of 11 patients and review of the literature. Dermatology. 2006;213(2):93–101.PubMedCrossRef

34.

Bischoff L, Derk CT. Eosinophilic fasciitis: demographics, disease pattern and response to treatment: report of 12 cases and review of the literature. Int J Dermatol. 2008;47(1):29–35.PubMedCrossRef

35.

Fujimoto M, Sato S, Ihn H, Kikuchi K, Yamada N, Takehara K. Serum aldolase level is a useful indicator of disease activity in eosinophilic fasciitis. J Rheumatol. 1995;22(3):563–5.PubMed

36.

Baumann F, Bruhlmann P, Andreisek G, Michel BA, Marincek B, Weishaupt D. MRI for diagnosis and monitoring of patients with eosinophilic fasciitis. AJR Am J Roentgenol. 2005;184(1):169–74.PubMed

37.

Barnes L, Rodnan GP, Medsger TA. Short D. Eosinophilic fasciitis. A pathologic study of twenty cases. Am J Pathol. 1979;96(2):493–518.PubMed

Title: Scleroderma Mimics
Authors: Jennifer Nashel
Virginia Steen
Publication date: 01-02-2012
Publisher: Current Science Inc.
Published in: Current Rheumatology Reports / Issue 1/2012
Print ISSN: 1523-3774
Electronic ISSN: 1534-6307
DOI: https://doi.org/10.1007/s11926-011-0220-8

ACC 2024 Congress Coverage

Heart Failure Video

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Scleroderma Mimics

Abstract

ACC 2024 Congress Coverage

Year in Review: Heart failure and cardiomyopathies

Semaglutide benefits people with type 2 diabetes and obesity-related heart failure

Incentivised to exercise

New intervention for STEMI-related cardiogenic shock

» View more highlights on the ACC 2024 congress hub page

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Please log in to get access to this content

Other articles of this Issue 1/2012

Angiogenesis and Vasculopathy in Systemic Sclerosis: Evolving Concepts

Drug-Induced Lupus Anticoagulants and Antiphospholipid Antibodies

An Update on the Evaluation and Management of Pulmonary Hypertension in Scleroderma

Laboratory Diagnosis of the Lupus Anticoagulant

Management of Obstetric Antiphospholipid Syndrome

Application of Biomarkers to Clinical Trials in Systemic Sclerosis

ACC 2024 Congress Coverage

Year in Review: Heart failure and cardiomyopathies

Semaglutide benefits people with type 2 diabetes and obesity-related heart failure

Incentivised to exercise

New intervention for STEMI-related cardiogenic shock

» View more highlights on the ACC 2024 congress hub page