Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress
Clinical Collections
Advances in Alzheimer’s

Keynote webinar | Spotlight on medication adherence

LIVE: Thursday 27th June 2024, 18:00-19:30 (CEST)
Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.
ADVANCED SEARCH
Log in
Top
Clinical Reviews in Allergy & Immunology
Published in: Clinical Reviews in Allergy & Immunology 3/2017

01-12-2017

Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review

Authors: Caterina Ferreli, Giulia Gasparini, Aurora Parodi, Emanuele Cozzani, Franco Rongioletti, Laura Atzori

Published in: Clinical Reviews in Allergy & Immunology | Issue 3/2017

Login to get access

Abstract

Scleroderma refers to an autoimmune connective tissue fibrosing disease, including three different subsets: localized scleroderma, limited cutaneous systemic sclerosis, and diffuse cutaneous systemic sclerosis with divergent patterns of organ involvement, autoantibody profiles, management, and prognostic implications. Although systemic sclerosis is considered the disease prototype that causes cutaneous sclerosis, there are many other conditions that can mimic and be confused with SSc. They can be classified into immune-mediated/inflammatory, immune-mediated/inflammatory with abnormal deposit (mucinoses), genetic, drug-induced and toxic, metabolic, panniculitis/vascular, and (para)neoplastic disorders according to clinico-pathological and pathogenetic correlations. This article reviews the clinical presentation with emphasis on cutaneous disease, etiopathogenesis, diagnosis, and treatment options available for the different forms of scleroderma firstly and for scleroderma-like disorders, including scleromyxedema, scleredema, nephrogenic systemic fibrosis, eosinophilic fasciitis, chronic graft-versus-host disease, porphyria cutanea tarda, diabetic stiff-hand syndrome (diabetic cheiroartropathy), and other minor forms. This latter group of conditions, termed also scleroderma mimics, sclerodermiform diseases, or pseudosclerodermas, shares the common thread of skin thickening but presents with distinct cutaneous manifestations, skin histology, and systemic implications or disease associations, differentiating each entity from the others and from scleroderma. The lack of Raynaud’s phenomenon, capillaroscopic abnormalities, or scleroderma-specific autoantibodies is also important diagnostic clues. As cutaneous involvement is the earliest, most frequent and characteristic manifestation of scleroderma and sclerodermoid disorders, dermatologists are often the first-line doctors who must be able to promptly recognize skin symptoms to provide the affected patient a correct diagnosis and appropriate management.
Literature
1.
2.
3.
Elhai M, Avouac J, Walker UA, Matucci-Cerinic M, Riemekasten G, Airo P et al (2016) A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: a EUSTAR prospective study. Ann Rheum Dis 75:163–169PubMedCrossRef
4.
Steen V, Domsic RT, Lucas M, Fertig N, Medsger TA Jr (2012) A clinical and serologic comparison of African American and Caucasian patients with systemic sclerosis. Arthritis Rheum 64:2986–2994PubMedPubMedCentralCrossRef
5.
Scalapino K, Arkachaisri T, Lucas M, Fertig N, Helfrich DJ, Londino AV Jr, Steen VD, Medsger TA Jr (2006) Childhood onset systemic sclerosis: classification, clinical and serologic features, and survival in comparison with adult onset disease. J Rheumatol 33:1004–1013PubMed
6.
7.
Gladman DD, Kung TN, Siannis F, Pellett F, Farewell VT, Lee P (2005) HLA markers for susceptibility and expression in scleroderma. J Rheumatol 32:1481–1487PubMed
8.
Radstake TR, Gorlova O, Rueda B et al (2010) Genome-wide association study of systemic sclerosis identifies CD247 as a new susceptibility locus. Nat Genet 42:426–429PubMedPubMedCentralCrossRef
9.
Distler O, Cozzio A (2016) Systemic sclerosis and localized scleroderma—current concepts and novel targets for therapy. Semin Immunopathol 38:87–95PubMedCrossRef
10.
11.
Grabell D, Hsieh C, Andrew R, Martires K, Kim A, Vasquez R et al (2014) The role of skin trauma in the distribution of morphea lesions: a cross-sectional survey of the morphea in adults and children cohort IV. J Am Acad 71:493–498CrossRef
12.
Matucci-Cerinic M, Kahaleh B, Wigley FM (2013) Evidence that systemic sclerosis is a vascular disease. Arthritis Rheum 65:1953–1962PubMedCrossRef
13.
Pattanaik D, Brown M, Postlethwaite AE (2011) Vascular involvement in systemic sclerosis (scleroderma). J Inflamm Res 4:105–125PubMedPubMedCentral
14.
Varga J (2008) Systemic sclerosis an update. Bull NYU Hosp Jt Dis 66:198–202PubMed
15.
16.
17.
Stern EP, Denton CP (2015) The pathogenesis of systemic sclerosis. Rheum Dis Clin N Am 41:367–382CrossRef
18.
van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A et al (2013) Classification criteria for systemic sclerosis: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Ann Rheum Dis 72:1747–1755PubMedCrossRef
19.
Jaeger VK, Wirz EG, Allanore Y, Rossbach P, Riemekasten G, Hachulla E, Distler O, Airò P, Carreira PE, Balbir Gurman A, Tikly M, Vettori S, Damjanov N, Müller-Ladner U, Distler JH, Li M, Walker UA, EUSTAR Co-authors (2016) Incidences and risk factors of organ manifestations in the early course of systemic sclerosis: a longitudinal EUSTAR study. PLoS One 11:e0163894PubMedPubMedCentralCrossRef
20.
21.
Meier FM, Frommer KW, Dinser R et al (2012) Update on the profile of the EUSTAR cohort: an analysis of the EULAR scleroderma trials and research group database. Ann Rheum Dis 71:1355e60CrossRef
22.
23.
Avouac J, Fransen J, Walker UA et al (2011) Preliminary criteria for the very early diagnosis of systemic sclerosis: results of a Delphi consensus study from EULAR scleroderma trials and research group. Ann Rheum Dis 70:476e81
24.
Minier T, Guiducci S, Bellando-Randone S, Bruni C, Lepri G, Czirjak L et al (2014) Preliminary analysis of the very early diagnosis of systemic sclerosis (VEDOSS) EUSTAR multicentre study: evidence for puffy fingers as a pivotal sign for suspicion of systemic sclerosis. Ann Rheum Dis 73:2087–2093PubMedCrossRef
25.
Clements PJ, Lachenbruch PA, Seibold JR, Zee B, Steen VD, Brennan P et al (1993) Skin thickness score in systemic sclerosis: an assessment of interobserver variability in 3 independent studies. J Rheumatol 20:1892–1896PubMed
26.
Barsotti S, Stagnaro C, Della Rossa A (2015) Systemic sclerosis: a critical digest of the recent literature. Clin Exp Rheumatol 33(Suppl. 91):S3–14PubMed
27.
Czirják L, Nagy Z, Aringer M et al (2007) The EUSTAR model for teaching and implementing the modified Rodnan skin score in systemic sclerosis. Ann Rheum Dis 66:966–969PubMedPubMedCentralCrossRef
28.
Hesselstrand R, Carlestam J, Wildt M, Sandqvist G, Andréasson K (2015) High frequency ultrasound of skin involvement in systemic sclerosis—a follow-up study. Arthritis Res Ther 17:329PubMedPubMedCentralCrossRef
29.
Santiago T, Alcacer-Pitarch B, Salvador MJ, Del Galdo F, Redmond AC, da Silva JAP (2016) A preliminary study using virtual touch imaging and quantification for the assessment of skin stiffness in systemic sclerosis. Clin Exp Rheumatol 34(Suppl. 100):S137–S141
30.
31.
Bajraktari IH, Kryeziu A, Sherifi F, Bajraktari H, Lahu A, Bajraktari G (2015) Oral manifestations of systemic sclerosis and correlation with anti-topoisomerase I antibodies (SCL-70). Med Arch 69:153–156PubMedPubMedCentralCrossRef
32.
Maddali Bongi S, Del Rosso A, Galluccio F, Tai G, Sigismondi F, Passalacqua M, Landi G, Baccini M, Conforti ML, Miniati I et al (2009) Efficacy of a tailored rehabilitation program for systemic sclerosis. Clin Exp Rheumatol 27:S44–S50
33.
Silvestre-Rangil J, Martinez-Herrera M, Silvestre FJ (2015) Dental management of patients with microstomia. A review of the literature and update on the treatment. J Oral Res 4:340–350CrossRef
34.
Crincoli V, Fatone L, Fanelli M, Rotolo RP, Chialà A, Favia G, Lapadula G (2016) Orofacial manifestations and temporomandibular disorders of systemic scleroderma: an observational study. Int J Mol Sci 17:1189PubMedCentralCrossRef
35.
Jagadish R, Mehta DS, Jagadish P (2012) Oral and periodontal manifestations associated with systemic sclerosis: a case series and review. J Indian Soc Periodontol 16:271–274PubMedPubMedCentralCrossRef
36.
Aliko A, Ciancaglini R, Alushi A, Tafaj A, Ruci D (2011) Temporomandibular joint involvement in rheumatoid arthritis, systemic lupus erythematosus and systemic sclerosis. Int J Oral Maxillofac Surg 40:704–709PubMedCrossRef
37.
Denton CP (2015) Systemic sclerosis: from pathogenesis to targeted therapy. Clin Exp Rheumatol 33:S3–S7PubMed
38.
Pizzorni C, Giampetruzzi AR, Mondino C, Facchiano A, Abeni D, Paolino S, Ruaro B, Smith V, Sulli A, Cutolo M (2016) Nailfold capillaroscopic parameters and skin telangiectasia patterns in patients with systemic sclerosis. Microvasc Res 111:20–24PubMedCrossRef
39.
Smith V, Decuman S, Sulli A, Bonroy C, Piettte Y, Deschepper E et al (2012) Do worsening scleroderma capillaroscopic pattern predict future severe organ involvement? A pilot study. Ann Rheum Dis 71:1636–1639PubMedCrossRef
40.
41.
42.
Nabil PA, Rao RA, Shrutakirti Shenoi D, Balachandran C (2006) Nail unit in collagen vascular diseases: a clinical, histopathological and direct immunofluorescence study. Indian J Dermatol 51:265–268CrossRef
43.
Tunc SE, Ertam I, Pirildar T, Turk T, Ozturk M, Doganavsargil E (2007) Nail changes in connective tissue diseases: do nail changes provide clues for the diagnosis? J Eur Acad Dermatol Venereol 21:497–503PubMed
44.
Sultan-Bichat N, Menard J, Perceau G, Staerman F, Bernard P, Reguia Z (2012) Treatment of calcinosis cutis by extracorporeal shock-wave lithotripsy. J Am Acad Dermatol 66:424–429PubMedCrossRef
45.
Johnstone EM, Hutchinson CE, Vail A, Chevance A, Herrick AL (2012) Acro-osteolysis in systemic sclerosis is associated with digital ischemia and severe calcinosis. Rheumatology 51:2234–2238PubMedCrossRef
46.
Avouac J, Mogavero G, Guerini H et al (2011) Predictive factors of hand radiographic lesions in systemic sclerosis: a prospective study. Ann Rheum Dis 70:630–633PubMedCrossRef
47.
Koutaissoff S, Vanthuyne M, Smith V et al (2011) Hand radiological damage in systemic sclerosis: comparison with a control group and clinical and functional correlations. Semin Arthritis Rheum 40:455–460PubMedCrossRef
48.
Giampetruzzi AR, Mondino C, Facchiano A, Bono R, Puddu P, Didona B, Abeni D, Cutolo M (2013) Association of dermoscopic profiles of telangiectases with nailfold videocapillaroscopic patterns in patients with systemic sclerosis. J Rheumatol 40:1630–1632PubMedCrossRef
49.
Hurabielle C, Avouac J, Lepri G, de Risi T, Kahan A, Allanore Y (2016) Skin telangiectasia and the identification of a subset of systemic sclerosis patients with severe vascular disease. Arthritis Care Res 68:1021–1027CrossRef
50.
Matucci-Cerinic M, Denton CP, Furst DE, Mayes MD, Hsu VM, Carpentier P et al (2011) Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial. Ann Rheum Dis 70:32–38PubMedCrossRef
51.
Walker UA, Tyndall A, Czirjak L, Denton C, Farge-Bancel D, Kowal-Bielecka O et al (2007) Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR scleroderma trials and research group database. Ann Rheum Dis 66:754–763PubMedPubMedCentralCrossRef
52.
Almeida C, Almeida I, Vasconcelos C (2015) Quality of life in systemic sclerosis. Auto Immun Rev 14:1087–1096CrossRef
53.
Denton CP, Krieg T, Guillevin L, Schwierin B, Rosenberg D, Silkey M et al (2012) De- mographic, clinical and antibody characteristics of patients with digital ulcers in systemic sclerosis: data from the DUO registry. Ann Rheum Dis 71:718–721PubMedPubMedCentralCrossRef
54.
Brand M, Hollaender R, Rosenberg D, Scott M, Hunsche E, Tyndall A, Denaro V, EUSTAR Co-investigators et al (2015) An observational cohort study of patients with newly diagnosed digital ulcer disease secondary to systemic sclerosis registered in the EUSTAR database. Clin Exp Rheumatol 33(4 Suppl 91):S47–S54PubMed
55.
Mihai C, Landewe R, van der Heijde D, Walker UA, Constantin PI, Gherghe AM et al (2016) Digital ulcers predict a worse disease course in patients with systemic sclerosis. Ann Rheum Dis Ann Rheum Dis 75:681–686PubMedCrossRef
56.
Cutolo M, Sulli A, Secchi ME, Paolino S, Pizzorni C (2006) Nailfold capillaroscopy is useful for the diagnosis and follow-up of autoimmune rheumatic diseases. A future tool for the analysis of microvascular heart involvement? Rheumatology 45:43–46CrossRef
57.
Sebastiani M, Manfredi A, Colaci M, D’amico R, Malagoli V, Giuggioli D et al (2009) Capillaroscopic skin ulcer risk index: a new prognostic tool for digital skin ulcer development in systemic sclerosis patients. Arthritis Rheum 61:688–694PubMedCrossRef
58.
Ruaro B, Sulli A, Smith V, Paolino S, Pizzorni C, Cutolo M (2015) Short-term follow-up of digital ulcers by laser speckle contrast analysis in systemic sclerosis patients. Microvasc Res 101:82–85PubMedCrossRef
59.
Barbano B, Marra AM, Quarta S, Gigante A, Barilaro G, Gasperini ML, Rosato E (2016) In systemic sclerosis skin perfusion of hands is reduced and may predict the occurrence of new digital ulcers. Microvasc Res 110:1–4PubMedCrossRef
60.
Anderson ME, Moore TL, Lunt M, Herrick AL (2007) The ‘distal-dorsal difference’: a thermographic parameter by which to differentiate between primary and secondary Raynaud’s phenomenon. Rheumatology 46:533–538PubMedCrossRef
61.
Onishi S, Homma Y, Hasegawa H, Yasukawa M (2013) Multiple tumoral calcinosis in systemic sclerosis. Intern Med 52:2689PubMedCrossRef
62.
Valenzuela A, Chung L (2015) Calcinosis: pathophysiology and management. Curr Opin Rheumatol 27:542–548PubMedCrossRef
63.
Zimmermann AF, Pizzichini MM (2013) Update on etiopathogenesis of systemic sclerosis. Rev Bras Reumatol 53:516–524PubMedCrossRef
64.
65.
Ee HL, Tan SH (2005) Reticulate hyperpigmented scleroderma: a new pigmentary manifestation. Clin Exp Dermatol 30:131–133PubMedCrossRef
66.
Ziemek J, Man A, Hinchcliff M, Varga J, Simms RW, Lafyatis R (2016) The relationship between skin symptoms and the scleroderma modification of the health assessment questionnaire, the modified Rodnan skin score, and skin pathology in patients with systemic sclerosis. Rheumatology 55:911–917PubMedCrossRef
67.
68.
Rongioletti F, Gambini C, Micalizzi C, Pastorino A, Rebora A (1994) Mucin deposits in morphea and systemic scleroderma. Dermatology 189:157–158PubMedCrossRef
69.
Fett N, Werth VP (2011) Update on morphea: part I. Epidemiology, clinical presentation, and pathogenesis. J Am Acad Dermatol 64:217–228PubMedCrossRef
70.
Kreuter A, Krieg T, Worm M et al (2009) AWMF guideline no. 013/066. Diagnosis and therapy of circumscribed scleroderma. J Dtsch Dermatol Ges 7:1–14
71.
Ho KT, Reveille JD (2003) The clinical relevance of autoantibodies in scleroderma. Arthritis Res Ther 5:80–93PubMed
72.
Kelly A, Derk CT (2015) Anti-RNA polymerase III antibodies in systemic sclerosis. Open J Rheumatol Autoimmune Dis 5:81–86CrossRef
73.
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 35:2439–2444PubMedCrossRef
74.
Walker KM, Pope J, participating members of the Scleroderma Clinical Trials Consortium (SCTC), Canadian Scleroderma Research Group (CSRG) (2012) Treatment of systemic sclerosis complications: what to use when first-line treatment fails—a consensus of systemic sclerosis experts. Semin Arthritis Rheum 42:42–55PubMedCrossRef
75.
Pope JE, Bellamy N, Seibold JR et al (2001) A randomized, controlled trial of methotrexate versus placebo in early diffuse scleroderma. Arthritis Rheum 44:1351–1358PubMedCrossRef
76.
77.
Tashkin DP, Elashoff R, Clements PJ, the Scleroderma Lung Study Research Group (2006) Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 354:2655–2666PubMedCrossRef
78.
Connolly KL, Griffith JL, McEvoy M, Lim HW (2015) Ultraviolet A1 phototherapy beyond morphea: experience in 83 patients. Photodermatol Photoimmunol Photomed 31:289–295PubMedCrossRef
79.
Kowal-Bielecka O, Landewé R, Avouac J, Chwiesko S, Miniati I et al (2009) EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR scleroderma trials and research group (EUSTAR). Ann Rheum Dis 68:620–628PubMedCrossRef
80.
Mouthon L, Bussone G, Berezné A, Noël LH, Guillevin L (2014) Scleroderma renal crisis. J Rheumatol 41:1040–1048PubMedCrossRef
81.
Papp G, Horvath IF, Barath S et al (2012) Immunomodulatory effects of extracorporeal photochemotherapy in systemic sclerosis. Clin Immunol 142:150–159PubMedCrossRef
82.
Knobler R, Berlin G, Calzavara-Pinton P et al (2014) Guidelines on the use of extracorporeal photopheresis. J Eur Acad Dermatol Venereol 28:1–37PubMedCrossRef
83.
Thompson AE, Shea B, Welch V, Fenlon D, Pope JE (2001) Calcium-channel blockers for Raynaud’s phenomenon in systemic sclerosis. Arthritis Rheum 44:1841–1847PubMedCrossRef
84.
Hughes M, Ong VH, Anderson ME et al (2015) Consensus best practice pathway of the UK scleroderma study group: digital vasculopathy in systemic sclerosis. Rheumatology 54:2015–2024PubMedCrossRef
85.
Fries R, Shariat K, von Wilmowsky H, Bohm M (2005) Sildenafil in the treatment of Raynaud’s phenomenon resistant to vasodilatory therapy. Circulation 112:2980–2985PubMed
86.
Foti R, Visalli E, Amato G et al (2017) Long-term clinical stabilization of scleroderma patients treated with a chronic and intensive IV iloprost regimen. Rheumatol Int 37:245–249PubMedCrossRef
87.
Tingey T, Shu J, Smuczek J et al (2013) Meta-analysis of healing and prevention of digital ulcers in systemic sclerosis. Arthritis Care Res (Hoboken) 65:1460–1471CrossRef
88.
De Cata A, Inglese M, Molinaro F et al (2016) Digital ulcers in scleroderma patients: a retrospective observational study. Int J Immunopathol Pharmacol 29:180–187PubMedCrossRef
89.
Balin SJ, Wetter DA, Andersen LK, Davis MD (2012) Calcinosis cutis occurring in association with autoimmune connective tissue disease: the Mayo Clinic experience with 78 patients, 1996–2009. Arch Dermatol 148:455–462PubMedCrossRef
90.
Gutierrez A Jr, Wetter DA (2012) Calcinosis cutis in autoimmune connective tissue diseases. Dermatol Ther 25:195–206PubMedCrossRef
91.
Dinsdale G, Murray A, Moore T et al (2014) A comparison of intense pulsed light and laser treatment of telangiectases in patients with systemic sclerosis: a within-subject randomized trial. Rheumatology 53:1422–1430PubMedCrossRef
92.
93.
Sanges S, Rivière S, Mekinian A et al (2017) Intravenous immunoglobulins in systemic sclerosis: data from a French nationwide cohort of 46 patients and review of the literature. Autoimmun Rev 16:377–384PubMedCrossRef
94.
Lee JJ, Pope JE (2016) Emerging drugs and therapeutics for systemic sclerosis. Expert Opin Emerg Drugs 21:421–430PubMedCrossRef
95.
Tyndall AJ, Bannert B, Vonk M et al (2010) Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis 69:1809PubMedCrossRef
96.
Rubio-Rivas M, Royo C, Simeón CP, Corbella X, Fonollosa V (2014) Mortality and survival in systemic sclerosis: systematic review and meta-analysis. Semin Arthritis Rheum 44:208–219PubMedCrossRef
97.
98.
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 24:73–80PubMed
99.
Zulian F, Athreya BH, Laxer R, Nelson AM, Feitosa de Oliveira SK, Punaro MG et al (2006) Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford) 45:614–620CrossRef
100.
Murray KJ, Laxer RM (2002) Scleroderma in children and adolescents. Rheum Dis Clin N Am 28:603–624CrossRef
101.
Christen-Zaech S, Hakim MD, Afsar FS, Paller AS (2008) Pediatric morphea (localized scleroderma): review of 136 patients. J Am Acad Dermatol 59:385–396PubMedCrossRef
102.
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 145:545–550PubMedPubMedCentralCrossRef
103.
Marzano AV, Menni S, Parodi A, Borghi A, Fuligni A, Fabbri P et al (2003) Localized scleroderma in adults and children. Clinical and laboratory investigations on 239 cases. Eur J Dermatol 13:171–176PubMed
104.
Zulian F, Cuffaro G, Sperotto F (2013) Scleroderma in children: an update. Curr Opin Rheumatol 25:643–650PubMedCrossRef
105.
Slobodin G, Rimar D (2017) Regulatory T cells in systemic sclerosis: a comprehensive review. Clin Rev Allergy Immunol 52:194–201PubMedCrossRef
106.
Peterson LS, Nelson AM, Su WP (1995) Classification of morphea (localized scleroderma). Mayo Clin Proc 70:1068–1076PubMedCrossRef
107.
Zulian F, Vallongo C, Woo P, Russo R, Ruperto N, Harper J et al (2005) Localized scleroderma in childhood is not just a skin disease. Arthritis Rheum 52:2873–2881PubMedCrossRef
108.
109.
Avancini J, Valente NY, Romiti R (2015) Generalized lenticular atrophoderma of Pasini and Pierini. Pediatr Dermatol 32:389–391PubMedCrossRef
110.
Zheng Y, Zhang R, Kang D, Zhu W (2015) Atrophoderma of Pasini and Pierini in zosteriform distribution. G Ital Dermatol Venereol 150:753–755PubMed
111.
Tas B, Sar M (2015) Linear atrophoderma of Moulin: a distinct entity or blaschko-linear variant of atrophoderma of Pasini and Pierini? G Ital Dermatol Venereol 150:636–638PubMed
112.
Zahedi Niaki O, Sissons W, Nguyen VH, Zargham R, Jafarian F (2015) Linear atrophoderma of Moulin: an underrecognized entity. Pediatr Rheumatol Online J 13:39PubMedPubMedCentralCrossRef
113.
114.
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 8:292–295PubMedCrossRef
115.
Kirsner RS, Pardes JB, Falanga V (1993) Solitary fibrosing paraspinal plaque: solitary morphea profunda. Br J Dermatol 128:99–101PubMedCrossRef
116.
Azad J, Dawn G, Shaffrali FC, Holmes SC, Barnetson RJ, Forsyth A (2004) Does solitary morphea profunda progress? Clin Exp Dermatol 29:25–27PubMedCrossRef
117.
Blaszczyk M, Krysicka-Janiger K, Jabłońska S (2000) Primary atrophic profound linear scleroderma. Dermatology 200:63–66PubMedCrossRef
118.
Malandrini A, Dotti MT, Federico A (1997) Selective ipsilateral neuromuscular involvement in a case of facial and somatic hemiatrophy. Muscle Nerve 20:890–892PubMedCrossRef
119.
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) 62:213–218CrossRef
120.
Hatzis JA, Stratigos AJ, Dimopoulos JC, Tzermias CK, Orfanidou A, Bassioukas KC (1992) Linear scleroderma with severe leg deformity. Australas J Dermatol 33:155–157PubMedCrossRef
121.
122.
Chiang KL, Chang KP, Wong TT, Hsu TR (2009) Linear scleroderma “en coup de sabre”: initial presentation as intractable partial seizures in a child. Pediatr Neonatol 50:294–298PubMedCrossRef
123.
Tollefson MM, Witman PM (2007) En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. J Am Acad Dermatol 56:257–263PubMedCrossRef
124.
Chung MH, Sum J, Morrell MJ, Horoupian DS (1995) Intracerebral involvement in scleroderma en coup de sabre: report of case with neuropathologic findings. Ann Neurol 37:679–681PubMedCrossRef
125.
Lüer W, Jöckel D, Henze T, Schipper HI (1990) Progressive inflammatory lesions of the brain parenchyma in localized scleroderma of the head. J Neurol 237:379–381PubMedCrossRef
126.
Jappe U, Hölzle E, Ring J (1996) Parry Romberg syndrome. Review and new observations based on a case with unusual features. Hautarzt 47:599–603PubMedCrossRef
127.
Jablonska S, Blaszczyk M, Rosinska D (1998) Progressive facial hemiatrophy and sclero- derma en coup de sabre: clinical presentation and course as related to the onset in early childhood and young adults. Arch Argent Dermatol 48:125–128
128.
Lehmann TJA (1992) The Parry-Romberg syndrome of progressive facial hemiatrophy and linear scleroderma en coup de sabre. Mistaken diagnosis or overlaping conditions? J Rheumatol 19:844–845
129.
DeFelipe J, Segura T, Arellano JI, Merchán A, DeFelipe-Oroquieta J, Martín P et al (2001) Neuropathological findings in a patient with epilepsy and the Parry-Romberg syndrome. Epilepsia 42:1198–1203PubMedCrossRef
130.
Woolfenden AR, Tong DC, Norbash AM, Albers GW (1998) Progressive facial hemiatrophy: abnormality of intracranial vasculature. Neurology 50:1915–1917PubMedCrossRef
131.
Gambichler T, Kreuter A, Hoffmann K, Bechara FG, Altmeyer P, Jansen T (2001) Billateral linear scleroderma “en coup de sabre” associated with facial atrophy and neurological complications. BMC Dermatol 1:9PubMedPubMedCentralCrossRef
132.
Wartenberg R (1945) Progressive facial hemiatrophy. Arch Neurol Psychiatr 54:75–96CrossRef
133.
Wolf HG, Ehrenclou AH (1927) Trophic disorders of central origin: report of a case of progressive facial hemiatrophy associated with a lipodistrophy and other metabolic derangements. JAMA 88:991–994CrossRef
134.
Parry CH (1825) Collections from unpublished papers, vol 178. Underwood, London, p 47
135.
Blaszczyk M, Królicki L, Krasu M, Glinska O, Jablonska S (2003) Progressive facial hemi- atrophy: central nervous system involvement and relationship with scleroderma en coup de sabre. J Rheumatol 30:1997–2004PubMed
136.
Blaszczyk M, Jablonska S (1999) Linear scleroderma en coup de sabre. Relationship with progressive facial hemiatrophy (PFH). Adv Exp Med Biol 455:101–104PubMedCrossRef
137.
Sánchez-Pérez S, Escandell-González I, Pinazo-Canales MI, Jordá-Cuevas E (2017) Bullous morphea: description of a new case and discussion of etiologic and pathogenic factors in bulla formation. Actas Dermosifiliogr 108:75–76PubMedCrossRef
138.
Fernández Flores A, Gatica Torres M, Tinoco Fragoso F, García Hidalgo F, García Hidalgo L, Monroy E et al (2015) Three cases of bullous morphea: histopathologic findings with implications regarding pathogenesis. J Cutan Pathol 42:144–149PubMedCrossRef
139.
Martinez SB, Gargallo V, Romero FT, Martinez LA, Peralto JL, Martín RL (2016) Generalized morphea with development of bullous lesions at the site of surgical scars. J Dtsch Dermatol Ges 14:827–829
140.
Qu T, Fang K (2014) Bullous morphea arising at the site of a healed herpes zoster. J Dermatol 41:553–554PubMedCrossRef
141.
Falanga V, Medsger TA Jr, Reichlin M (1987) Antinuclear and anti-single-stranded DNA antibodies in morphea and generalized morphea. Arch Dermatol 123:350–353PubMedCrossRef
142.
Arkachaisri T, Pino S (2008) Localized scleroderma severity index and global assessments: a pilot study of outcome instruments. J Rheumatol 35:650–657PubMedCrossRef
143.
Kreuter A, Krieg T, Worm M, Wenzel J, Moinzadeh P, Kuhn A, Aberer E, Scharffetter-Kochanek K, Horneff G, Reil E, Weberschock T, Hunzelmann N (2016) German guidelines for the diagnosis and therapy of localized scleroderma. J Dtsch Dermatol Ges 14:199–216PubMedCrossRef
144.
Cunningham BB, Landells ID, Langman C et al (1998) Topical calcipotriolo for morphea/linear scleroderma. J Am Acad Dermatol 39:211–215PubMedCrossRef
145.
Stefanaki C, Stefanaki K, Kontochristopoulos G et al (2008) Topical tacrolimus 0.1% ointment in the treatment of localized scleroderma. An open label clinical and histological study. J Dermatol 35:712–718PubMedCrossRef
146.
Kroft EB, Groeneveld TJ, Seyger MM, de Jong EM (2009) Efficacy of topical tacrolimus 0.1% in active plaque morphea: randomized, double-blind, emollient-controlled pilot study. Am J Clin Dermatol 10:181–187PubMedCrossRef
147.
Kroft EB, Creemers MC, van den Hoogen FH, Boezeman JB, de Jong EM (2009) 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 160:1075–1082PubMedCrossRef
148.
Li SC, Torok KS, Pope E et al (2012) Development of consensus treatment plans for juvenile localized scleroderma: a roadmap toward comparative effectiveness studies in juvenile localized scleroderma. Arthritis Care Res (Hoboken) 64:1175–1185
149.
Mertens JS, Marsman D, van de Kerkhof PC, Hoppenreijs EP, Knaapen HK, Radstake TR, de Jong EM, Seyger MM (2016) Use of mycophenolate Mofetil in patients with severe localized scleroderma resistant or intolerant to methotrexate. Acta Derm Venereol 96:510–513PubMedCrossRef
150.
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. Arch Dermatol 130:663–664PubMedCrossRef
151.
Wright NA, Mazori DR, Patel M, Merola JF, Femia AN, Vleugels RA (2016) Epidemiology and treatment of eosinophilic fasciitis: an analysis of 63 patients from 3 tertiary care centers. JAMA Dermatol 152:97–99PubMedCrossRef
152.
Rongioletti F (2006) Lichen myxedematosus (papular mucinosis): new concepts and perspectives for an old disease. Semin Cutan Med Surg 25:100–104PubMedCrossRef
153.
Rongioletti F, Merlo G, Cinotti E et al (2013) Scleromyxedema: a multicenter study of characteristics, comorbidities, course, and therapy in 30 patients. J Am Acad Dermatol 69:66PubMedCrossRef
154.
155.
Rongioletti F, Cattarini G, Sottofattori E, Rebora A (2003) Granulomatous reaction after intradermal injections of hyaluronic acid gel. Arch Dermatol 139:815–816PubMedCrossRef
156.
157.
Rongioletti F, Merlo G, Carli C et al (2016) Histopathologic characteristics of scleromyxedema: a study of a series of 34 cases. J Am Acad Dermatol 74:1194–1200PubMedCrossRef
158.
Rongioletti F, Cozzani E, Parodi A (2010) Scleromyxedema with an interstitial granulomatous-like pattern: a rare histologic variant mimicking granuloma annulare. J Cutan Pathol 37:1084–1087PubMedCrossRef
159.
Bidier M, Zschoche C, Gholam P et al (2012) Scleromyxoedema: clinical follow-up after successful treatment with high-dose immunoglobulins reveals different long-term outcomes. Acta Derm Venereol 92:408PubMedCrossRef
160.
161.
Blum M, Wigley FM, Hummers LK (2008) Scleromyxedema: a case series highlighting long-term outcomes of treatment with intravenous immunoglobulin (IVIG). Medicine (Baltimore) 87:10–20CrossRef
162.
Rey JB, Luria RB (2009) Treatment of scleromyxedema and dermatoneuro syndrome with intravenous immunoglobulin. J Am Acad Dermatol 60:1037–1041PubMedCrossRef
163.
Bidier M, Zschoche C, Gholam P et al (2012) Scleromyxoedema: clinical follow-up after successful treatment with high-dose immunoglobulins reveals different long-term outcomes. Acta Derm Venereol 92:408–409PubMedCrossRef
164.
Sansbury JC, Cocuroccia B, Jorizzo JL et al (2004) Treatment of recalcitrant scleromyxedema with thalidomide in 3 patients. J Am Acad Dermatol 51:126–131PubMedCrossRef
165.
Efthimiou P, Blanco M (2008) Intravenous gammaglobulin and thalidomide may be an effective therapeutic combination in refractory scleromyxedema: case report and discussion of the literature. Semin Arthritis Rheum 38:188–194PubMedCrossRef
166.
Bos R, de Waal EG, Kuiper H et al (2011) Thalidomide and dexamethasone followed by autologous stem cell transplantation for scleromyxoedema. Rheumatology (Oxford) 50:1925–1926CrossRef
167.
Cañueto J, Labrador J, Román C et al (2012) The combination of bortezomib and dexamethasone is an efficient therapy for relapsed/refractory scleromyxedema: a rare disease with new clinical insights. Eur J Haematol 88:450PubMedCrossRef
168.
Horn KB, Horn MA, Swan J et al (2004) A complete and durable clinical response to high-dose dexamethasone in a patient with scleromyxedema. J Am Acad Dermatol 51:S120–S123PubMedCrossRef
169.
Rongioletti F, Kaiser F, Cinotti E et al (2015) Scleredema. A multicentre study of characteristics, comorbidities, course and therapy in 44 patients. J Eur Acad Dermatol Venereol 29:2399–2404PubMedCrossRef
170.
Beers WH, Ince A, Moore TL (2006) Scleredema adultorum of Buschke: a case report and review of the literature. Semin Arthritis Rheum 35:355–359PubMedCrossRef
171.
Lewerenz V, Ruzicka T (2007) Scleredema adultorum associated with type 2 diabetes mellitus: a report of three cases. J Eur Acad Dermatol Venereol 21:560–561PubMed
172.
Varga J, Gotta S, Li L, Sollberg S, Di Leonardo M (1995) Scleredema adultorum: case report and demonstration of abnormal expression of extracellular matrix genes in skin fibroblasts in vivo and in vitro. Br J Dermatol 132:992–999PubMedCrossRef
173.
Ioannidou DI, Krasagakis K, Stefanidou MP et al (2001) Scleredema adultorum of Buschke presenting as periorbital edema: a diagnostic challenge. J Am Acad Dermatol 65:41–44
174.
Morais P, Almeida M, Santos P, Azevedo F (2011) Scleredema of Buschke following mycoplasma pneumoniae respiratory infection. Int J Dermatol 50:454–457PubMedCrossRef
175.
Rongioletti F, Rebora A (2001) Cutaneous mucinoses: microscopic criteria for diagnosis. Am J Dermatopathol 23:257–267PubMedCrossRef
176.
Miyares FJ, Kuriakose R, Deleu DT, El-Wahad NA, Al-Hail H (2008) Scleredema diabeticorum with unusual presentation and fatal outcome. Indian J Dermatol 53:217–219PubMedPubMedCentralCrossRef
177.
Xiao T, Yang ZH, He CD, Chen HD (2007) Scleredema adultorum treated with narrow-band ultraviolet B phototherapy. J Dermatol 34:270–272PubMedCrossRef
178.
Yüksek J, Sezer E, Köseoğlu D, Markoç F, Yıldız H (2010) Scleredema treated with broad-band ultraviolet a phototherapy plus colchicine. Photodermatol Photoimmunol Photomed 26:257–260PubMedCrossRef
179.
Szturz P, Adam Z, Vašků V, Feit J, Krejčí M, Pour L, Hájek R, Mayer J (2013) Complete remission of multiple myeloma associated scleredema after bortezomib-based treatment. Leuk Lymphoma 54:1324–1326PubMedCrossRef
180.
Lee FY, Chiu HY, Chiu HC (2011) Treatment of acquired reactive perforating collagenosis with allopurinol incidentally improves scleredema diabeticorum. J Am Acad Dermatol 65:e115–e117PubMedCrossRef
181.
Stables GI, Taylor PC, Highet AS (2000) Scleredema associated with paraproteinaemia treated by extracorporeal photopheresis. Br J Dermatol 142:781–783PubMedCrossRef
182.
Bowen AR, Smith L, Zone JJ (2003) Scleredema adultorum of Buschke treated with radiation. Arch Dematol 139:780–784CrossRef
183.
Aichelburg MC, Loewe R, Schicher N, Sator PG, Karlhofer FM, Stingl G, Jalili A (2012) Successful treatment of poststreptococcal scleredema adultorum Buschke with intravenous immunoglobulins. Arch Dermatol 148:1126–1128PubMedCrossRef
184.
Cowper SE, Robin HS, Steinberg SM, Su LD, Gupta S, LeBoit PE (2000) Scleromyxoedema-like cutaneous diseases in renal-dialysis patients. Lancet 356:1000–1001PubMedCrossRef
185.
Girardi M, Kay J, Elston DM, LeBoit PE, Abu-Alfa A, Cowper SE (2011) Nephrogenic systemic fibrosis: clinicopathological definition and workup recommendations. J Am Acad Dermatol 65:1095–1106.e7PubMedCrossRef
186.
Knopp EA, Cowper SE (2008) Nephrogenic systemic fibrosis: early recognition and treatment. Semin Dial 21:123–128PubMedCrossRef
187.
Thomsen HS, Marckmann P (2008) Extracellular Gd-CA: differences in prevalence of NSF. Eur J Radiol 66:180–183PubMedCrossRef
188.
Thomsen HS, Morcos SK, Almen T et al (2013) Nephrogenic systemic fibrosis and gadolinium-based contrast media: updated ESUR contrast medium safety committee guidelines. Eur Radiol 23:307–318PubMedCrossRef
189.
Jan F, Segal JM, Dyer J, LeBoit P, Siegfried E, Frieden IJ (2003) Nephrogenic fibrosing dermopathy: two pediatric cases. J Pediatr 143:678–681PubMedCrossRef
190.
Perazella MA, Rodby RA (2007) Gadolinium use in patients with kidney disease: a cause for concern. Semin Dial 20:179–185PubMedCrossRef
191.
Newton BB, Jimenez SA (2009) Mechanism of NSF: new evidence challenging the prevailing theory. J Magn Reson Imaging 30:1277–1283PubMedCrossRef
192.
Cowper SE (2003) Nephrogenic fibrosing dermopathy: the first 6 years. Curr Opin Rheumatol 15:785–790PubMedCrossRef
193.
Larson KN, Gagnon AL, Darling MD, Patterson JW, Cropley TG (2015) Nephrogenic systemic fibrosis manifesting a decade after exposure to gadolinium. JAMA Dermatol 151:1117–1120PubMedCrossRef
194.
195.
Swaminathan S, High WA, Ranville J, Horn TD, Hiatt K, Thomas M et al (2008) Cardiac and vascular metal deposition with high mortality in nephrogenic systemic fibrosis. Kidney Int 73:1413–1418PubMedCrossRef
196.
High WA, Ayers RA, Chandler J et al (2007) Gadolinium is detectable within the tissue of patients with nephrogenic systemic fibrosis. J Am Acad Dermatol 56:21PubMedCrossRef
197.
198.
Bhawan J, Perez-Chua TA, Goldberg L (2013) Sclerotic bodies beyond nephrogenic systemic fibrosis. J Cutan Pathol 40:812–817PubMedCrossRef
199.
Boyd AS, Zic JA, Abraham JL (2007) Gadolinium deposition in nephrogenic fibrosing dermopathy. J Am Acad Dermatol 56:27PubMedCrossRef
200.
High WA, Ayers RA, Cowper SE (2007) Gadolinium is quantifiable within the tissue of patients with nephrogenic systemic fibrosis. J Am Acad Dermatol 56:710PubMedCrossRef
201.
Igreja AC, Mesquita Kde C, Cowper SE, Costa IM (2012) Nephrogenic systemic fibrosis: concepts and perspective. An Bras Dermatol 87:597–607PubMedCrossRef
202.
Elmholdt TR, Buus NH, Ramsing M, Olesen AB (2013) Antifibrotic effect after low-dose imatinib mesylate treatment in patients with nephrogenic systemic fibrosis: an open-label non-randomized, uncontrolled clinical trial. J Eur Acad Dermatol Venereol 27:779–784PubMedCrossRef
203.
Mendoza FA, Artlett CM, Sandorfi N et al (2006) Description of 12 cases of nephrogenic fibrosing dermopathy and review of the literature. Semin Arthritis Rheum 35:238PubMedPubMedCentralCrossRef
204.
Cuffy MC, Singh M, Formica R et al (2011) Renal transplantation for nephrogenic systemic fibrosis: a case report and review of the literature. Nephrol Dial Transplant 26:1099–1101PubMedCrossRef
205.
206.
Shulman LE (1974) Diffuse fasciite with hypergammaglobulinemia and eosinophilia: a new syndrome? J Rheumatol 1:46–49
207.
Lebeaux D, Sène D (2012) Eosinophilic fasciitis (Shulman disease). Best Pract Res Clin Rheumatol 26:449–458PubMedCrossRef
208.
209.
Sène D (2015) Eosinophilic fasciitis (Shulman’s disease): diagnostic and therapeutic review. Rev Med Interne 36:738–745PubMedCrossRef
210.
Pinal-Fernandez I, Selva-O’ Callaghan A, Grau JM (2014) Diagnosis and classification of eosinophilic fasciitis. Autoimmun Rev 13:379–3824PubMedCrossRef
211.
Endo Y, Tamura A, Matsushima Y, Iwasaki T, Hasegawa M, Nagai Y, Ishikawa O (2007) Eosinophilic fasciitis: report of two cases and a systematic review of the literature dealing with clinical variables that predict outcome. Clin Rheumatol 26:1445–1451PubMedCrossRef
212.
Bischoff L, Derk CT (2008) Eosinophilic fasciitis: demographics, disease pattern and response to treatment: report of 12 cases and review of the literature. Int J Dermatol 47:29–35PubMedCrossRef
213.
Toquet C, Hamidou MA, Renaudin K, Jarry A, Foulc P, Barbarot S, Laboisse C, Mussini JM (2003) In situ immunophenotype of the inflammatory infiltrate in eosinophilic fasciitis. J Rheumatol 30:1811–1815PubMed
214.
Asano Y, Ihn H, Jinnin M, Tamaki Z, Tamaki K, Sato S (2014) Serum levels of matrix metalloproteinase-13 in patients with eosinophilic fasciitis. J Dermatol 41:746–748PubMedCrossRef
215.
Daniel RS, Lavery S, Maize JC Jr, Brown AN, Bolster MB (2009) Unilateral eosinophilic fasciitis: an under-recognized subtype? J Clin Rheumatol 15:247–249PubMedCrossRef
216.
Patterson J (2016) Disorders of collagen. In: Weedon’s skin pathology, 4th edn. Elsevier, pp 348–379
217.
Nashel J, Steen V (2015) The use of an elevated aldolase in diagnosing and managing eosinophilic fasciitis. Clin Rheumatol 34:1481–1484PubMedCrossRef
218.
Jinnin M, Ihn H, Yamane K, Asano Y, Yazawa N, Tamaki K (2004) Serum levels of tissue inhibitor of metalloproteinase-1 and 2 in patients with eosinophilic fasciitis. Br J Dermatol 151:407–412PubMedCrossRef
219.
Dybowski F, Neuen-Jacob E, Braun J (2008) Eosinophilic fasciitis and myositis: use of imaging modalities for diagnosis and monitoring. Ann Rheum Dis 67:572–574PubMedCrossRef
220.
Kurimoto R, Ikeda K, Nakagomi D, Nakajima H (2016) Eosinophilic fasciitis illustrated by [(18)F] FDG-PET/CT. Intern Med 55:2321–2322PubMedCrossRef
221.
Mondal S, Goswami RP, Sinha D, Ghosh A (2015) Ultrasound is a useful adjunct in diagnosis of eosinophilic fasciitis. Rheumatology 54:2041PubMedCrossRef
222.
Oza VS, Walsh R, North J, Berger TG, Murase JE (2016) Treatment of eosinophilic fasciitis with Sirolimus. JAMA Dermatol 152:488–490PubMedCrossRef
223.
Mertens JS, Zweers MC, Kievit W, Knaapen HK, Gerritsen M, Radstake TR, van den Hoogen FH, Creemers MC, de Jong EM (2016) High-dose intravenous pulse methotrexate in patients with eosinophilic fasciitis. JAMA Dermatol 152:1262–1265PubMedCrossRef
224.
Kroft EB, Berkhof NJ, van de Kerkhof PC, Gerritsen RM, de Jong EM (2008) Ultraviolet a phototherapy for sclerotic skin diseases: a systematic review. J Am Acad Dermatol 59:1017–1030PubMedCrossRef
225.
Romano C, Rubegni P, De Aloe G, Stanghellini E, D’Ascenzo G, Andreassi L, Fimiani M (2003) Extracorporeal photochemotherapy in the treatment of eosinophilic fasciitis. J Eur Acad Dermatol Venereol 17:10–13PubMedCrossRef
226.
Hymes SR, Alousi AM, Cowen EW (2012) Graft-versus-host disease: part I. Pathogenesis and clinical manifestations of graft-versus-host disease. J Am Acad Dermatol 66:515.e1–515.18CrossRef
227.
Chosidow O, Bagot M, Vernant JP, Roujeau JC, Cordonnier C, Kuentz M, Wechsler J, André C, Touraine R, Revuz J (1992) Sclerodermatous chronic graft-versus-host disease. Analysis of seven cases. J Am Acad Dermatol 26:49–55PubMedCrossRef
228.
Zhang J, Chen F, Ueki T, Date H (2015) Imatinib for sclerodermatous graft-versus-host disease in lung transplantation. Interact Cardiovasc Thorac Surg 21:260–262PubMedCrossRef
229.
Lazzeri L, Tripo L, Pescitelli L, Ricceri F, Prignano F (2016) A pediatric case of Sclerodermatous graft-versus-host disease responsive to ultraviolet A1 phototherapy. Pediatr Dermatol 33:e99–102PubMedCrossRef
230.
Bisaccia E, Palangio M, Gonzalez J (2011) Long-term extracorporeal photochemotherapy in a pediatric patient with refractory sclerodermatous chronic graft-versus-host disease. Transfus Apher Sci 45:187–190PubMedCrossRef
231.
Bell SA, Faust H, Mittermüller J, Kolb HJ, Meurer M (1996) Specificity of antinuclear antibodies in scleroderma-like chronic graft-versus-host disease: clinical correlation and histocompatibility locus antigen association. Br J Dermatol 134:848–854PubMedCrossRef
232.
233.
White JM, Devereux S, Pagliuca A, Salisbury JR, du Vivier AW, Creamer D (2006) Koebnerizing sclerodermatous graft-versus-host disease caused by donor lymphocyte infusion and interferon-alpha. Br J Dermatol 155:621–623PubMedCrossRef
234.
Delaney TA, Morehouse C, Brohawn PZ, Groves C, Colonna M, Yao Y, Sanjuan M, Coyle AJ (2016) Type I IFNs regulate inflammation, vasculopathy, and fibrosis in chronic cutaneous graft-versus-host disease. J Immunol 197:42–50PubMedCrossRef
235.
236.
Kharfan-Dabaja MA, Mhaskar AR, Djulbegovic B, Cutler C, Mohty M, Kumar A (2009) Efficacy of rituximab in the setting of steroid-refractory chronic graft-versus-host disease: a systematic review and meta-analysis. Biol Blood Marrow Transplant 15:1005–1013PubMedCrossRef
237.
White JM, Creamer D, du Vivier AW, Pagliuca A, Ho AY, Devereux S, Salisbury JR, Mufti GJ (2007) Sclerodermatous graft-versus-host disease: clinical spectrum and therapeutic challenges. Br J Dermatol 156:1032–1038PubMedCrossRef
238.
Peñas PF, Jones-Caballero M, Aragués M, Fernández-Herrera J, Fraga J, Garcia-Diez A (2002) Sclerodermatous graft-vs-host disease. Clinical and pathological study of 17 patients. Arch Dermatol 138:924–934PubMedCrossRef
239.
Schaffer JV, McNiff JM, Seropian S, Cooper DL, Bolognia JL (2005) Lichen sclerosus and eosinophilic fasciitis as manifestations of chronic graft-versus-host disease: expanding the sclerodermoid spectrum. J Am Acad Dermatol 53:591–601PubMedCrossRef
240.
Jachiet M, de Masson A, Peffault de Latour R, Rybojad M, Robin M, Bourhis JH, Xhaard A, Dhedin N, Sicre de Fontbrune F, Suarez F, Barete S, Parquet N, Nguyen S, Ades L, Rubio MT, Wittnebel S, Bagot M, Socié G, Bouaziz JD (2014) Skin ulcers related to chronic graft-versus-host disease: clinical findings and associated morbidity. Br J Dermatol 171:63–68PubMedCrossRef
241.
Akay BN, Sanli H, Topcuoglu P, Arat M, Akyol A (2010) Nailfold capillary abnormalities are prevalent in sclerodermoid graft-versus-host disease and readily detected with dermatoscopy. Br J Dermatol 162:1076–1082PubMedCrossRef
242.
Man J, Man J, Kalisiak M, Birchall IW, Salopek TG (2010) Chronic cutaneous graft-versus-host disease manifesting as calcinosis cutis universalis on a background of widespread sclerodermatoid changes. J Cutan Med Surg 14:249–253PubMedCrossRef
243.
Osmola-Mańkowska A, Silny W, Dańczak-Pazdrowska A, Polańska A, Olek-Hrab K, Sadowska-Przytocka A, Żaba R, Jenerowicz D (2013) Assessment of chronic sclerodermoid graft-versus-host disease patients, using 20 MHz high-frequency ultrasonography and cutometer methods. Skin Res Technol 19:e417–e422PubMedCrossRef
244.
Carcagnì MR, De Aloe G, D’Ascenzo G, Rubegni P, Fimiani M (2008) Extracorporeal photopheresis in graft-versus-host disease. J Dtsch Dermatol Ges 6:451–457PubMedCrossRef
245.
Calzavara Pinton P, Porta F, Izzi T, Venturini M, Capezzera R, Zane C, Notarangelo LD (2003) Prospects for ultraviolet A1 phototherapy as a treatment for chronic cutaneous graft-versus-host disease. Haematologica 88:1169–1175PubMed
246.
Lazar J, Poonawalla T, Teng JM (2011) A case of sclerodermatous graft-versus-host disease responsive to imatinib therapy. Pediatr Dermatol 28:172–175PubMedCrossRef
247.
Pines M, Snyder D, Yarkoni S, Nagler A (2003) Halofuginone to treat fibrosis in chronic graft-versus-host disease and scleroderma. Biol Blood Marrow Transplant 9:417–425PubMedCrossRef
248.
249.
Amezyane T, Abouzahir A, Fatihi J, Sekkach Y, Mahassin F, Sedrati O, Ghafir D, Ohayon V (2010) A sclerodermiform porphyria cutanea tarda. Intern Med 49:205–206PubMedCrossRef
250.
Kranzelbinder B, Wiednig M, El-Shabrawi-Caelen L, Aberer W, Aberer E (2015) Sclerodermiform porphyria cutanea tarda after torasemide. Eur J Dermatol 25:484–485PubMed
251.
Wallaeys E, Thierling U, Lang E, Neumann NJ, Frank J (2014) Porphyria cutanea tarda with sclerodermatous changes and hemochromatosis. Hautarzt 65:272–274PubMedCrossRef
252.
Lim HW (1989) Mechanisms of phototoxicity in porphyria cutanea tarda and erythropoietic protoporphyria. Immunol Ser 46:671–685PubMed
253.
Torinuki W, Kudoh K, Tagami H (1989) Increased mast cell numbers in the sclerotic skin of Porphyria Cutanea Tarda. Dermatologica 178:75–78PubMedCrossRef
254.
Thomas CL, Badminton MN, Rendall JR, Anstey AV (2008) Sclerodermatous changes of face, neck and scalp associated with familial porphyria cutanea tarda. Clin Exp Dermatol 33:422–442PubMedCrossRef
255.
Khayat R, Dupuy A, Pansé I, Bagot M, Cordoliani F (2013) Sclerodermatous changes in porphyria cutanea tarda: six cases. Ann Dermatol Venereol 140:589–597PubMedCrossRef
256.
Zemtsov R, Zemtsov A (2010) Porphyria cutanea tarda presenting as scleroderma. Cutis 85:203–205PubMed
257.
Bulaj ZJ, Phillips JD, Ajioka RS, Franklin MR, Griffen LM, Guinee DJ et al (2000) Hemochromatosis genes and other factors contributing to the pathogenesis of porphyria cutanea tarda. Blood 95:1565–1571PubMed
258.
Vieira FM, Aoki V, Oliveira ZN, Martins JE (2010) Study of direct immunofluorescence, immunofluorescence mapping and light microscopy in porphyria cutanea tarda. An Bras Dermatol 85:827–837PubMedCrossRef
259.
Singal AK, Kormos-Hallberg C, Lee C, Sadagoparamanujam VM, Grady JJ, Freeman DH Jr, Anderson KE (2012) Low-dose hydroxychloroquine is as effective as phlebotomy in treatment of patients with porphyria cutanea tarda. Clin Gastroenterol Hepatol 10:1402–1409PubMedPubMedCentralCrossRef
260.
Singal AK, Kormos-Hallberg C, Lee C et al (2012) Low-dose hydroxychloroquine is as effective as phlebotomy in treatment of patients with porphyria cutanea tarda. Clin Gastroenterol Hepatol 10:1402PubMedPubMedCentralCrossRef
261.
Monastirli A, Georgiou S, Bolsen K et al (1999) Treatment of porphyria cutanea tarda with oral thalidomide. Skin Pharmacol Appl Ski Physiol 12:305CrossRef
262.
Ayres S Jr, Mihan R (1978) Porphyria cutanea tarda: response to vitamin E. A review and two case reports. Cutis 22:50PubMed
263.
Ballantyne JA, Hooper G (2004) The hand and diabetes. Curr Orthop 18:118–125CrossRef
264.
Ravindran Rajendran S, Bhansali A, Walia R et al (2011) Prevalence and pattern of hand soft-tissue changes in type 2 diabetes mellitus. Diabetes Metab 37:312–317PubMedCrossRef
265.
Chen LH, Li C-Y, Kuo LC, Wang L-Y, Kuo KN, Jou IM, Hou W-H (2015) Risk of hand syndromes in patients with diabetes mellitus. A population-based cohort study in Taiwan. Medicine 94:e1575PubMedPubMedCentralCrossRef
266.
Al-Matubsi HY, Hamdan F, Alhanbali OA et al (2011) Diabetic hand syndromes as a clinical and diagnostic tool for diabetes mellitus patients. Diabetes Res Clin Pract 94:225–229PubMedCrossRef
267.
Somai P (2011) Limited joint mobility in diabetes mellitus: the clinical implications. J Musculoskel Med 28:118–124
268.
Ishibashi Y, Matsui T, Maeda S, Higashimoto Y, Yamagishi S (2013) Advanced glycation end products evoke endothelial cell damage by stimulating soluble dipeptidyl peptidase-4 production and its interaction with mannose 6-phosphate/insulin-like growth factor II receptor. Cardiovasc Diabetol 12:125PubMedPubMedCentralCrossRef
269.
Yokote K, Chanprasert S, Lee L, Eirich K, Takemoto M, Watanabe A et al (2017) WRN mutation update: mutation spectrum, patient registries, and translational prospects. Hum Mutat 38:7–15PubMedCrossRef
270.
Davis T, Brook AJ, Rokicki MJ, Bagley MC, Kipling D (2016) Evaluating the role of p38 MAPK in the accelerated cell senescence of Werner syndrome fibroblasts. Pharmaceuticals 9:23PubMedCentralCrossRef
271.
Goto M, Ishikawa Y, Sugimoto M, Furuichi Y (2013) Werner syndrome: a changing pattern of clinical manifestations in Japan (1917–2008). BioScience Trends 7:13–22PubMed
272.
273.
Lee YA, Stevens HP, Delaporte E, Wahn U, Reis A (2000) A gene for an autosomal dominant scleroatrophic syndrome predisposing to skin cancer (Huriez syndrome) maps to chromosome 4q23. Am J Hum Genet 66:326–330PubMedPubMedCentralCrossRef
274.
Massip L, Garand C, Paquet ER, Cogger VC, O’Reilly JN, Tworek L, Hatherell A, Taylor CG, Thorin E, Zahradka P, Le Couteur DG, Lebel M (2010) Vitamin C restores healthy aging in a mouse model for Werner syndrome. FASEB J 24:158–172PubMedCrossRef
275.
Tokita M, Kennedy SR, Risques RA, Chun SG, Pritchard C, Oshima J, Liu Y, Bryant-Greenwood PK, Welcsh P, Monnat RJ (2016) Werner syndrome through the lens of tissue and tumour genomics. Sci Rep 6:32038PubMedPubMedCentralCrossRef
276.
Kikuchi K, Hoashi T, Yazawa N, Tamaki K (2006) Pseudoscleroderma associated with cancer. Clin Exp Dermatol 31:381–383PubMedCrossRef
277.
Juarez M, Marshall R, Denton C, Evely R (2008) Paraneoplastic scleroderma secondary to hairy cell leukaemia successfully treated with cladribine. Rheumatology 47:1734–1735PubMedCrossRef
278.
Querfeld C, Sollberg S, Huerkamp C, Eckes B, Krieg T (2000) Pseudoscleroderma associated with lung cancer: correlation of collagen typ 1 and connective tissue growth factor gene expression. Br J Dermatol 142:1228–1233PubMedCrossRef
279.
Jedlickova H, Durčanská V, Vašků V (2016) Paraneoplastic scleroderma: are there any clues? Acta Dermatovenerol Croat 24:78–80PubMed
280.
281.
Allen JA, Peterson A, Sufit R et al (2011) Post-epidemic eosinophilia-myalgia syndrome associated with L-tryptophan. Arthritis Rheum 63:3633–3639PubMedCrossRef
282.
Rongioletti F, Rebora A (1992) Cutaneous toxic mucinosis. J Am Acad Dermatol 26:788–790CrossRef
283.
D’Cruz D (2000) Autoimmune diseases associated with drugs, chemicals and environmental factors. Toxicol Lett 112:421–432PubMedCrossRef
284.
285.
Kaufman LD, Gruber BL, Gomez-Reino JJ et al (1994) Fibrogenic growth factors in the eosinophilia–myalgia syndrome and the toxic oil syndrome. Arch Dermatol 130:41–47PubMedCrossRef
286.
Sharma SK, Handa R, Sood R et al (2004) Bleomycin-induced scleroderma. J Assoc Physicians India 52:76–77PubMed
287.
Niklas K, Niklas AA, Majewski D, Puszczewicz M (2016) Rheumatic diseases induced by drugs and environmental factors: the state-of-the-art—part one. Reumatologia 54:122–127PubMedPubMedCentralCrossRef
Metadata
Title
Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review
Authors
Caterina Ferreli
Giulia Gasparini
Aurora Parodi
Emanuele Cozzani
Franco Rongioletti
Laura Atzori
Publication date
01-12-2017
Publisher
Springer US
Published in
Clinical Reviews in Allergy & Immunology / Issue 3/2017
Print ISSN: 1080-0549
Electronic ISSN: 1559-0267
DOI
https://doi.org/10.1007/s12016-017-8625-4

Other articles of this Issue 3/2017

Clinical Reviews in Allergy & Immunology 3/2017 Go to the issue

ReviewPaper

Cutaneous Manifestations of Medium- and Large-Vessel Vasculitis

ReviewPaper

Cutaneous and Mucosal Manifestations of Sjögren’s Syndrome

ReviewPaper

Cutaneous Manifestations of Dermatomyositis: a Comprehensive Review

ReviewPaper

Skin Manifestations of Rheumatoid Arthritis, Juvenile Idiopathic Arthritis, and Spondyloarthritides

ReviewPaper

Neonatal Systemic Lupus Erythematosus Syndrome: a Comprehensive Review

ReviewPaper

Cutaneous Manifestations of ANCA-Associated Small Vessels Vasculitis
Live Webinar | 27-06-2024 | 18:00 (CEST)

Keynote webinar | Spotlight on medication adherence

Reserve your place

Live: Thursday 27th June 2024, 18:00-19:30 (CEST)

WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.

Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.

Prof. Kevin Dolgin
Prof. Florian Limbourg
Prof. Anoop Chauhan
Developed by: Springer Medicine
Obesity Clinical Trial Summary

At a glance: The STEP trials

Read more

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine
Image Credits