Differentiating Scleroderma from other diseases: Difference between revisions

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==Overview==
==Overview==
Scleroderma must be differentiated from other diseases that cause [[skin]] thickening, [[sclerodactyly]], [[edema]] and symptoms of [[GERD]] such as scleredema, [[scleromyxedema]], [[eosinophilic fasciitis]], chronic [[graft-versus-host disease]], drug induced scleroderma, scleroderma overlap syndromes, [[diabetic]] cheiroarthropathy, [[myxedema]] and [[nephrogenic systemic fibrosis]].
Scleroderma must be differentiated from other diseases that cause [[skin]] thickening, [[sclerodactyly]], [[edema]], and symptoms of [[GERD]] such as scleredema, [[scleromyxedema]], [[eosinophilic fasciitis]], chronic [[graft-versus-host disease]], drug induced scleroderma, scleroderma overlap syndromes, [[diabetic]] cheiroarthropathy, [[myxedema]], and [[nephrogenic systemic fibrosis]].


==Differentiating Scleroderma from other Diseases==
==Differentiating Scleroderma from other Diseases==
Scleroderma must be differentiated from other diseases that cause [[skin]] thickening, [[sclerodactyly]], [[edema]] and symptoms of [[GERD]] such as scleredema, [[scleromyxedema]], [[eosinophilic fasciitis]], chronic [[graft-versus-host disease]], drug induced scleroderma, scleroderma overlap syndromes, [[diabetic]] cheiroarthropathy, [[myxedema]] and [[nephrogenic systemic fibrosis]].
Scleroderma must be differentiated from other diseases that cause [[skin]] thickening, [[sclerodactyly]], [[edema]], and symptoms of [[GERD]] such as scleredema, [[scleromyxedema]], [[eosinophilic fasciitis]], chronic [[graft-versus-host disease]], drug induced scleroderma, scleroderma overlap syndromes, [[diabetic]] cheiroarthropathy, [[myxedema]], and [[nephrogenic systemic fibrosis]].


===Differentiating Scleroderma from other Diseases===
===Differentiating Scleroderma from other Diseases===
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! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Blood indices
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Blood indices
|-
|-
| rowspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Scleroderma<ref name="pmid3361530">{{cite journal |vauthors=LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA, Rowell N, Wollheim F |title=Scleroderma (systemic sclerosis): classification, subsets and pathogenesis |journal=J. Rheumatol. |volume=15 |issue=2 |pages=202–5 |date=February 1988 |pmid=3361530 |doi= |url=}}</ref><ref name="pmid8340733">{{cite journal |vauthors=Black CM |title=Scleroderma--clinical aspects |journal=J. Intern. Med. |volume=234 |issue=2 |pages=115–8 |date=August 1993 |pmid=8340733 |doi= |url=}}</ref>
| rowspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Scleroderma'''<ref name="pmid3361530">{{cite journal |vauthors=LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA, Rowell N, Wollheim F |title=Scleroderma (systemic sclerosis): classification, subsets and pathogenesis |journal=J. Rheumatol. |volume=15 |issue=2 |pages=202–5 |date=February 1988 |pmid=3361530 |doi= |url=}}</ref><ref name="pmid8340733">{{cite journal |vauthors=Black CM |title=Scleroderma--clinical aspects |journal=J. Intern. Med. |volume=234 |issue=2 |pages=115–8 |date=August 1993 |pmid=8340733 |doi= |url=}}</ref>
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Limited cutaneous [[systemic sclerosis]] ([[CREST syndrome]])
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Limited cutaneous [[systemic sclerosis]] ([[CREST syndrome]])'''
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([[induration]])
([[induration]])
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* [[Dyspnea]]
* [[Dyspnea]]
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Diffuse cutaneous [[systemic sclerosis]]
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Diffuse cutaneous [[systemic sclerosis]]'''
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([[induration]])
([[induration]])
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* [[Dyspnea]]
* [[Dyspnea]]
|-
|-
| rowspan="5" style="background: #DCDCDC; padding: 5px; text-align: center;" |Systemic diseases
| rowspan="5" style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Systemic diseases'''
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Scleredema
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Scleredema'''
(Buschke's disease)<ref name="pmid26304054">{{cite journal |vauthors=Rongioletti F, Kaiser F, Cinotti E, Metze D, Battistella M, Calzavara-Pinton PG, Damevska K, Girolomoni G, André J, Perrot JL, Kempf W, Cavelier-Balloy B |title=Scleredema. A multicentre study of characteristics, comorbidities, course and therapy in 44 patients |journal=J Eur Acad Dermatol Venereol |volume=29 |issue=12 |pages=2399–404 |date=December 2015 |pmid=26304054 |doi=10.1111/jdv.13272 |url=}}</ref>
'''(Buschke's disease)<ref name="pmid26304054">{{cite journal |vauthors=Rongioletti F, Kaiser F, Cinotti E, Metze D, Battistella M, Calzavara-Pinton PG, Damevska K, Girolomoni G, André J, Perrot JL, Kempf W, Cavelier-Balloy B |title=Scleredema. A multicentre study of characteristics, comorbidities, course and therapy in 44 patients |journal=J Eur Acad Dermatol Venereol |volume=29 |issue=12 |pages=2399–404 |date=December 2015 |pmid=26304054 |doi=10.1111/jdv.13272 |url=}}</ref>'''
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* N/A
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* [[Fasting blood sugar]]
* [[Fasting blood sugar]]
* [[HbA1C]]
* [[HbA1C]]
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* N/A
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* Normal [[epidermis]]
* Normal [[epidermis]]
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* Fingers are spared
* Fingers are spared
* Immobility of shoulders and chest
* Immobility of shoulders and chest
* Associated with [[URTI|viral URTI]] and [[Diabetes mellitus type 1]], [[monoclonal gammopathy]]
* Associated with [[URTI|viral URTI]] and [[diabetes mellitus type 1]], [[monoclonal gammopathy]]
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Scleromyxedema]]
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Scleromyxedema|'''Scleromyxedema''']]
([[lichen myxedematosus]])<ref name="pmid11174386">{{cite journal |vauthors=Rongioletti F, Rebora A |title=Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema |journal=J. Am. Acad. Dermatol. |volume=44 |issue=2 |pages=273–81 |date=February 2001 |pmid=11174386 |doi=10.1067/mjd.2001.111630 |url=}}</ref><ref name="pmid3336281">{{cite journal |vauthors=Gabriel SE, Perry HO, Oleson GB, Bowles CA |title=Scleromyxedema: a scleroderma-like disorder with systemic manifestations |journal=Medicine (Baltimore) |volume=67 |issue=1 |pages=58–65 |date=January 1988 |pmid=3336281 |doi= |url=}}</ref>
'''([[lichen myxedematosus]])'''<ref name="pmid11174386">{{cite journal |vauthors=Rongioletti F, Rebora A |title=Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema |journal=J. Am. Acad. Dermatol. |volume=44 |issue=2 |pages=273–81 |date=February 2001 |pmid=11174386 |doi=10.1067/mjd.2001.111630 |url=}}</ref><ref name="pmid3336281">{{cite journal |vauthors=Gabriel SE, Perry HO, Oleson GB, Bowles CA |title=Scleromyxedema: a scleroderma-like disorder with systemic manifestations |journal=Medicine (Baltimore) |volume=67 |issue=1 |pages=58–65 |date=January 1988 |pmid=3336281 |doi= |url=}}</ref>
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* N/A
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Monoclonal gammopathy]] (IgG-lambda)
* [[Monoclonal gammopathy]] (IgG-lambda)
* Normal [[TSH]]
* Normal [[TSH]]
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |
* N/A
| style="background: #F5F5F5; padding: 5px;" |
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* [[Fibroblast]] proliferation in the [[dermis]]
* [[Fibroblast]] proliferation in the [[dermis]]
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* [[Scalp]] and head are affected
* [[Scalp]] and head are affected
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Eosinophilic fasciitis]]<ref name="pmid2346160">{{cite journal |vauthors=Herson S, Brechignac S, Piette JC, Mouthon JM, Coutellier A, Bletry O, Godeau P |title=Capillary microscopy during eosinophilic fasciitis in 15 patients: distinction from systemic scleroderma |journal=Am. J. Med. |volume=88 |issue=6 |pages=598–600 |date=June 1990 |pmid=2346160 |doi= |url=}}</ref><ref name="pmid3668010">{{cite journal |vauthors=Falanga V, Medsger TA |title=Frequency, levels, and significance of blood eosinophilia in systemic sclerosis, localized scleroderma, and eosinophilic fasciitis |journal=J. Am. Acad. Dermatol. |volume=17 |issue=4 |pages=648–56 |date=October 1987 |pmid=3668010 |doi= |url=}}</ref>
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Eosinophilic fasciitis|'''Eosinophilic fasciitis''']]<ref name="pmid2346160">{{cite journal |vauthors=Herson S, Brechignac S, Piette JC, Mouthon JM, Coutellier A, Bletry O, Godeau P |title=Capillary microscopy during eosinophilic fasciitis in 15 patients: distinction from systemic scleroderma |journal=Am. J. Med. |volume=88 |issue=6 |pages=598–600 |date=June 1990 |pmid=2346160 |doi= |url=}}</ref><ref name="pmid3668010">{{cite journal |vauthors=Falanga V, Medsger TA |title=Frequency, levels, and significance of blood eosinophilia in systemic sclerosis, localized scleroderma, and eosinophilic fasciitis |journal=J. Am. Acad. Dermatol. |volume=17 |issue=4 |pages=648–56 |date=October 1987 |pmid=3668010 |doi= |url=}}</ref>
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| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |
* N/A
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Eosinophilia]] ([[peripheral blood smear]])
* [[Eosinophilia]] ([[peripheral blood smear]])
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* Hands and feet are not involved
* Hands and feet are not involved
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Chronic [[graft-versus-host disease]]<ref name="pmid16198778">{{cite journal |vauthors=Schaffer JV, McNiff JM, Seropian S, Cooper DL, Bolognia JL |title=Lichen sclerosus and eosinophilic fasciitis as manifestations of chronic graft-versus-host disease: expanding the sclerodermoid spectrum |journal=J. Am. Acad. Dermatol. |volume=53 |issue=4 |pages=591–601 |date=October 2005 |pmid=16198778 |doi=10.1016/j.jaad.2005.06.015 |url=}}</ref><ref name="pmid21791415">{{cite journal |vauthors=Martires KJ, Baird K, Steinberg SM, Grkovic L, Joe GO, Williams KM, Mitchell SA, Datiles M, Hakim FT, Pavletic SZ, Cowen EW |title=Sclerotic-type chronic GVHD of the skin: clinical risk factors, laboratory markers, and burden of disease |journal=Blood |volume=118 |issue=15 |pages=4250–7 |date=October 2011 |pmid=21791415 |pmc=3204741 |doi=10.1182/blood-2011-04-350249 |url=}}</ref>
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Chronic [[graft-versus-host disease]]'''<ref name="pmid16198778">{{cite journal |vauthors=Schaffer JV, McNiff JM, Seropian S, Cooper DL, Bolognia JL |title=Lichen sclerosus and eosinophilic fasciitis as manifestations of chronic graft-versus-host disease: expanding the sclerodermoid spectrum |journal=J. Am. Acad. Dermatol. |volume=53 |issue=4 |pages=591–601 |date=October 2005 |pmid=16198778 |doi=10.1016/j.jaad.2005.06.015 |url=}}</ref><ref name="pmid21791415">{{cite journal |vauthors=Martires KJ, Baird K, Steinberg SM, Grkovic L, Joe GO, Williams KM, Mitchell SA, Datiles M, Hakim FT, Pavletic SZ, Cowen EW |title=Sclerotic-type chronic GVHD of the skin: clinical risk factors, laboratory markers, and burden of disease |journal=Blood |volume=118 |issue=15 |pages=4250–7 |date=October 2011 |pmid=21791415 |pmc=3204741 |doi=10.1182/blood-2011-04-350249 |url=}}</ref>
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
(induration)
(induration)
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| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Increased [[serum bilirubin]] concentration
* Increased [[serum bilirubin]] concentration
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |
* N/A
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Thick [[collagen]] bundles in the [[dermis]]
* Thick [[collagen]] bundles in the [[dermis]]
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* [[Esophageal]] sclerosis
* [[Esophageal]] sclerosis
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Drug induced scleroderma<ref name="pmid6160247">{{cite journal |vauthors=Finch WR, Rodnan GP, Buckingham RB, Prince RK, Winkelstein A |title=Bleomycin-induced scleroderma |journal=J. Rheumatol. |volume=7 |issue=5 |pages=651–9 |date=1980 |pmid=6160247 |doi= |url=}}</ref><ref name="pmid10524560">{{cite journal |vauthors=Passiu G, Cauli A, Atzeni F, Aledda M, Dessole G, Sanna G, Nurchis P, Vacca A, Garau P, Laudadio M, Mathieu A |title=Bleomycin-induced scleroderma: report of a case with a chronic course rather than the typical acute/subacute self-limiting form |journal=Clin. Rheumatol. |volume=18 |issue=5 |pages=422–4 |date=1999 |pmid=10524560 |doi= |url=}}</ref>
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Drug-induced scleroderma'''<ref name="pmid6160247">{{cite journal |vauthors=Finch WR, Rodnan GP, Buckingham RB, Prince RK, Winkelstein A |title=Bleomycin-induced scleroderma |journal=J. Rheumatol. |volume=7 |issue=5 |pages=651–9 |date=1980 |pmid=6160247 |doi= |url=}}</ref><ref name="pmid10524560">{{cite journal |vauthors=Passiu G, Cauli A, Atzeni F, Aledda M, Dessole G, Sanna G, Nurchis P, Vacca A, Garau P, Laudadio M, Mathieu A |title=Bleomycin-induced scleroderma: report of a case with a chronic course rather than the typical acute/subacute self-limiting form |journal=Clin. Rheumatol. |volume=18 |issue=5 |pages=422–4 |date=1999 |pmid=10524560 |doi= |url=}}</ref>
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| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" | +/-
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* N/A
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* N/A
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* N/A
| style="background: #F5F5F5; padding: 5px;" |
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* [[IgG]] and [[complement]] deposits on [[epidermal]] [[keratinocytes]]
* [[IgG]] and [[complement]] deposits on [[epidermal]] [[keratinocytes]]
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* Skin changes present on trunk and extremities
* Skin changes present on trunk and extremities
|-
|-
| rowspan="3" style="background: #DCDCDC; padding: 5px; text-align: center;" |Scleroderma overlap syndromes<ref name="pmid20477023">{{cite journal |vauthors=Satoh M, Chan EK, Sobel ES, Kimpel DL, Yamasaki Y, Narain S, Mansoor R, Reeves WH |title=Clinical implication of autoantibodies in patients with systemic rheumatic diseases |journal=Expert Rev Clin Immunol |volume=3 |issue=5 |pages=721–38 |date=September 2007 |pmid=20477023 |doi=10.1586/1744666X.3.5.721 |url=}}</ref><ref name="pmid24389298">{{cite journal |vauthors=Moinzadeh P, Aberer E, Ahmadi-Simab K, Blank N, Distler JH, Fierlbeck G, Genth E, Guenther C, Hein R, Henes J, Herich L, Herrgott I, Koetter I, Kreuter A, Krieg T, Kuhr K, Lorenz HM, Meier F, Melchers I, Mensing H, Mueller-Ladner U, Pfeiffer C, Riemekasten G, Sárdy M, Schmalzing M, Sunderkoetter C, Susok L, Tarner IH, Vaith P, Worm M, Wozel G, Zeidler G, Hunzelmann N |title=Disease progression in systemic sclerosis-overlap syndrome is significantly different from limited and diffuse cutaneous systemic sclerosis |journal=Ann. Rheum. Dis. |volume=74 |issue=4 |pages=730–7 |date=April 2015 |pmid=24389298 |pmc=4392314 |doi=10.1136/annrheumdis-2013-204487 |url=}}</ref><ref name="pmid27126733">{{cite journal |vauthors=Foocharoen C, Netwijitpan S, Mahakkanukrauh A, Suwannaroj S, Nanagara R |title=Clinical characteristics of scleroderma overlap syndromes: comparisons with pure scleroderma |journal=Int J Rheum Dis |volume=19 |issue=9 |pages=913–23 |date=September 2016 |pmid=27126733 |doi=10.1111/1756-185X.12884 |url=}}</ref><ref name="pmid21844148">{{cite journal |vauthors=Pakozdi A, Nihtyanova S, Moinzadeh P, Ong VH, Black CM, Denton CP |title=Clinical and serological hallmarks of systemic sclerosis overlap syndromes |journal=J. Rheumatol. |volume=38 |issue=11 |pages=2406–9 |date=November 2011 |pmid=21844148 |doi=10.3899/jrheum.101248 |url=}}</ref>
| rowspan="3" style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Scleroderma overlap syndromes'''<ref name="pmid20477023">{{cite journal |vauthors=Satoh M, Chan EK, Sobel ES, Kimpel DL, Yamasaki Y, Narain S, Mansoor R, Reeves WH |title=Clinical implication of autoantibodies in patients with systemic rheumatic diseases |journal=Expert Rev Clin Immunol |volume=3 |issue=5 |pages=721–38 |date=September 2007 |pmid=20477023 |doi=10.1586/1744666X.3.5.721 |url=}}</ref><ref name="pmid24389298">{{cite journal |vauthors=Moinzadeh P, Aberer E, Ahmadi-Simab K, Blank N, Distler JH, Fierlbeck G, Genth E, Guenther C, Hein R, Henes J, Herich L, Herrgott I, Koetter I, Kreuter A, Krieg T, Kuhr K, Lorenz HM, Meier F, Melchers I, Mensing H, Mueller-Ladner U, Pfeiffer C, Riemekasten G, Sárdy M, Schmalzing M, Sunderkoetter C, Susok L, Tarner IH, Vaith P, Worm M, Wozel G, Zeidler G, Hunzelmann N |title=Disease progression in systemic sclerosis-overlap syndrome is significantly different from limited and diffuse cutaneous systemic sclerosis |journal=Ann. Rheum. Dis. |volume=74 |issue=4 |pages=730–7 |date=April 2015 |pmid=24389298 |pmc=4392314 |doi=10.1136/annrheumdis-2013-204487 |url=}}</ref><ref name="pmid27126733">{{cite journal |vauthors=Foocharoen C, Netwijitpan S, Mahakkanukrauh A, Suwannaroj S, Nanagara R |title=Clinical characteristics of scleroderma overlap syndromes: comparisons with pure scleroderma |journal=Int J Rheum Dis |volume=19 |issue=9 |pages=913–23 |date=September 2016 |pmid=27126733 |doi=10.1111/1756-185X.12884 |url=}}</ref><ref name="pmid21844148">{{cite journal |vauthors=Pakozdi A, Nihtyanova S, Moinzadeh P, Ong VH, Black CM, Denton CP |title=Clinical and serological hallmarks of systemic sclerosis overlap syndromes |journal=J. Rheumatol. |volume=38 |issue=11 |pages=2406–9 |date=November 2011 |pmid=21844148 |doi=10.3899/jrheum.101248 |url=}}</ref>
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Scleroderma-[[systemic lupus erythematosus]] overlap
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Scleroderma-[[systemic lupus erythematosus]] overlap'''
| style="background: #F5F5F5; padding: 5px;" | +
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(rash)
(rash)
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* Decreased [[C3 (complement)|C3]], C4 and CH50
* Decreased [[C3 (complement)|C3]], C4 and CH50
* [[Anemia]]
* [[Anemia]]
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |
* N/A
| style="background: #F5F5F5; padding: 5px;" |
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* [[Inflammation]] at the [[dermal]]-[[epidermal]] junction
* [[Inflammation]] at the [[dermal]]-[[epidermal]] junction
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* [[Arthritis]]
* [[Arthritis]]
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Scleroderma-[[polymyositis]] overlap
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Scleroderma-[[polymyositis]] overlap'''
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(rash)
(rash)
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* [[Aldolase]]
* [[Aldolase]]
* [[Anemia]]
* [[Anemia]]
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |
* N/A
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Inflammation]] at the [[Dermis|dermal]]-[[epidermal]] junction
* [[Inflammation]] at the [[Dermis|dermal]]-[[epidermal]] junction
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* Symmetric [[proximal muscle weakness]]
* Symmetric [[proximal muscle weakness]]
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Scleroderma-[[rheumatoid arthritis]] overlap
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Scleroderma-[[rheumatoid arthritis]] overlap'''
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
(rash)
(rash)
Line 307: Line 318:
* Elevated [[ESR]]
* Elevated [[ESR]]
* [[Anemia]]
* [[Anemia]]
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |
* N/A
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Inflammation]] at the [[dermal]]-[[epidermal]] junction
* [[Inflammation]] at the [[dermal]]-[[epidermal]] junction
Line 316: Line 328:
* Clinical findings that suggest [[rheumatoid arthritis]] ([[RA]]) but do not fulfill the ACR criteria for [[RA]]<ref name="pmid208725952">{{cite journal |vauthors=Aletaha D, Neogi T, Silman AJ, Funovits J, Felson DT, Bingham CO, Birnbaum NS, Burmester GR, Bykerk VP, Cohen MD, Combe B, Costenbader KH, Dougados M, Emery P, Ferraccioli G, Hazes JM, Hobbs K, Huizinga TW, Kavanaugh A, Kay J, Kvien TK, Laing T, Mease P, Ménard HA, Moreland LW, Naden RL, Pincus T, Smolen JS, Stanislawska-Biernat E, Symmons D, Tak PP, Upchurch KS, Vencovský J, Wolfe F, Hawker G |title=2010 Rheumatoid arthritis classification criteria: an American College of Rheumatology/European League Against Rheumatism collaborative initiative |journal=Arthritis Rheum. |volume=62 |issue=9 |pages=2569–81 |date=September 2010 |pmid=20872595 |doi=10.1002/art.27584 |url=}}</ref>
* Clinical findings that suggest [[rheumatoid arthritis]] ([[RA]]) but do not fulfill the ACR criteria for [[RA]]<ref name="pmid208725952">{{cite journal |vauthors=Aletaha D, Neogi T, Silman AJ, Funovits J, Felson DT, Bingham CO, Birnbaum NS, Burmester GR, Bykerk VP, Cohen MD, Combe B, Costenbader KH, Dougados M, Emery P, Ferraccioli G, Hazes JM, Hobbs K, Huizinga TW, Kavanaugh A, Kay J, Kvien TK, Laing T, Mease P, Ménard HA, Moreland LW, Naden RL, Pincus T, Smolen JS, Stanislawska-Biernat E, Symmons D, Tak PP, Upchurch KS, Vencovský J, Wolfe F, Hawker G |title=2010 Rheumatoid arthritis classification criteria: an American College of Rheumatology/European League Against Rheumatism collaborative initiative |journal=Arthritis Rheum. |volume=62 |issue=9 |pages=2569–81 |date=September 2010 |pmid=20872595 |doi=10.1002/art.27584 |url=}}</ref>
|-
|-
| rowspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Endocrine disorders
| rowspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Endocrine disorders'''
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Diabetic]] cheiroarthropathy<ref name="pmid6753855">{{cite journal |vauthors=Seibold JR |title=Digital sclerosis in children with insulin-dependent diabetes mellitus |journal=Arthritis Rheum. |volume=25 |issue=11 |pages=1357–61 |date=November 1982 |pmid=6753855 |doi= |url=}}</ref><ref name="pmid8485952">{{cite journal |vauthors=Jelinek JE |title=The skin in diabetes |journal=Diabet. Med. |volume=10 |issue=3 |pages=201–13 |date=April 1993 |pmid=8485952 |doi= |url=}}</ref>
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''[[Diabetic]] cheiroarthropathy'''<ref name="pmid6753855">{{cite journal |vauthors=Seibold JR |title=Digital sclerosis in children with insulin-dependent diabetes mellitus |journal=Arthritis Rheum. |volume=25 |issue=11 |pages=1357–61 |date=November 1982 |pmid=6753855 |doi= |url=}}</ref><ref name="pmid8485952">{{cite journal |vauthors=Jelinek JE |title=The skin in diabetes |journal=Diabet. Med. |volume=10 |issue=3 |pages=201–13 |date=April 1993 |pmid=8485952 |doi= |url=}}</ref>
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +


Line 327: Line 339:
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |
* N/A
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Fasting blood sugar]] ≥126 mg/dL
* [[Fasting blood sugar]] ≥126 mg/dL
* [[HbA1C]] ≥ 6.5%
* [[HbA1C]] ≥ 6.5%
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" | -
* N/A
| style="background: #F5F5F5; padding: 5px;" |
* N/A
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Fasting blood sugar]] ≥126 mg/dL
* [[Fasting blood sugar]] ≥126 mg/dL
* [[HbA1C]] ≥ 6.5%
* [[HbA1C]] ≥ 6.5%
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Long standing [[Type I diabetes mellitus]]
* Long standing [[type I diabetes mellitus]]
* [[Skin]] thickening of fingers
* [[Skin]] thickening of fingers
* Flexion [[contractures]] on fingers
* Flexion [[contractures]] on fingers
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Myxedema]]<ref name="pmid1607406">{{cite journal |vauthors=Heymann WR |title=Cutaneous manifestations of thyroid disease |journal=J. Am. Acad. Dermatol. |volume=26 |issue=6 |pages=885–902 |date=June 1992 |pmid=1607406 |doi= |url=}}</ref>
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Myxedema|'''Myxedema''']]<ref name="pmid1607406">{{cite journal |vauthors=Heymann WR |title=Cutaneous manifestations of thyroid disease |journal=J. Am. Acad. Dermatol. |volume=26 |issue=6 |pages=885–902 |date=June 1992 |pmid=1607406 |doi= |url=}}</ref>
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +


Line 357: Line 372:
* [[Anemia]]
* [[Anemia]]
* Serum [[TSH]] elevated  
* Serum [[TSH]] elevated  
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |
* N/A
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Atrophied [[Epidermis (skin)|epidermis]]
* Atrophied [[Epidermis (skin)|epidermis]]
Line 366: Line 382:
* Nonpitting [[edema]] present
* Nonpitting [[edema]] present
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Renal diseases
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Renal diseases'''
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Nephrogenic systemic fibrosis]]<ref name="pmid17053507">{{cite journal |vauthors=Galan A, Cowper SE, Bucala R |title=Nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy) |journal=Curr Opin Rheumatol |volume=18 |issue=6 |pages=614–7 |date=November 2006 |pmid=17053507 |doi=10.1097/01.bor.0000245725.94887.8d |url=}}</ref>
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Nephrogenic systemic fibrosis|'''Nephrogenic systemic fibrosis''']]<ref name="pmid17053507">{{cite journal |vauthors=Galan A, Cowper SE, Bucala R |title=Nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy) |journal=Curr Opin Rheumatol |volume=18 |issue=6 |pages=614–7 |date=November 2006 |pmid=17053507 |doi=10.1097/01.bor.0000245725.94887.8d |url=}}</ref>
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
(induration)
(induration)
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| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |
* N/A
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Elevated [[C-reactive protein (CRP)|C-reactive protein]]
* Elevated [[C-reactive protein (CRP)|C-reactive protein]]
Line 382: Line 399:
* Decreased [[serum albumin]]
* Decreased [[serum albumin]]
* Decreased [[creatinine clearance]]
* Decreased [[creatinine clearance]]
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |
* N/A
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Fibrosis]] of [[dermis]]
* [[Fibrosis]] of [[dermis]]

Revision as of 13:26, 10 May 2018

Scleroderma Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: M. Khurram Afzal, MD [2]

Overview

Scleroderma must be differentiated from other diseases that cause skin thickening, sclerodactyly, edema, and symptoms of GERD such as scleredema, scleromyxedema, eosinophilic fasciitis, chronic graft-versus-host disease, drug induced scleroderma, scleroderma overlap syndromes, diabetic cheiroarthropathy, myxedema, and nephrogenic systemic fibrosis.

Differentiating Scleroderma from other Diseases

Scleroderma must be differentiated from other diseases that cause skin thickening, sclerodactyly, edema, and symptoms of GERD such as scleredema, scleromyxedema, eosinophilic fasciitis, chronic graft-versus-host disease, drug induced scleroderma, scleroderma overlap syndromes, diabetic cheiroarthropathy, myxedema, and nephrogenic systemic fibrosis.

Differentiating Scleroderma from other Diseases

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging Histopathology
Skin thickening Raynaud phenomenon Heart burn Edema (swelling) Sclerodactyly Telangiectasia Impaired mobility Autoantibodies Blood indices
Scleroderma[1][2] Limited cutaneous systemic sclerosis (CREST syndrome) +

(induration)

+ + +/- + + +/-
  • Nailfold microvascular changes on nailfold video capillaroscopy (NVC)[3]
  • Chest HRCT scan showing evidence of pulmonary fibrosis
  • Nailfold microvascular changes
  • Dilated capillary loops
  • Microhemmorhages
Diffuse cutaneous systemic sclerosis +

(induration)

+ + +/- + + +/-
  • Nailfold microvascular changes on nailfold video capillaroscopy (NVC)[5]
  • Chest HRCT scan showing evidence of pulmonary fibrosis
  • Nailfold microvascular changes
  • Dilated capillary loops
  • Microhemmorhages
Systemic diseases Scleredema

(Buschke's disease)[6]

+ - - + - - +
  • N/A
  • N/A
Scleromyxedema

(lichen myxedematosus)[7][8]

+

(waxy yellow-red papules)

+/- +/- + - - -
  • N/A
  • N/A
Eosinophilic fasciitis[9][10] +

(orange peel-peau d'orange appearance)

- - + - - -
  • N/A
  • Normal appearance on nailfold video capillaroscopy (NVC)
  • Fasciitis present on the trunk sparing extremities
  • Visible collapse of superficial veins when the limb is elevated
  • Hands and feet are not involved
Chronic graft-versus-host disease[11][12] +

(induration)

- - - - - +
  • N/A
Drug-induced scleroderma[13][14] + + +/- +/- + +/- -
  • N/A
  • N/A
  • N/A
Scleroderma overlap syndromes[15][16][17][18] Scleroderma-systemic lupus erythematosus overlap +

(rash)

+ + +/- + + +/-
  • N/A
Scleroderma-polymyositis overlap +

(rash)

+ + +/- + + +/-
  • Anti-Ro52 antibody
  • Antinuclear antibody (ANA)
  • Anti-Jo-1 antibody
  • Anti-SRP antibody
  • Anti-Mi-2 antibody
  • Anti-centromere antibody (ACA)
  • Anti-topoisomerase-I (Scl-70) antibody
  • Anti-RNA polymerase III antibody
  • N/A
Scleroderma-rheumatoid arthritis overlap +

(rash)

+ + +/- + + +/-
  • N/A
  • Anti-Ro52 antibody
  • Anti-CCP antibody
Endocrine disorders Diabetic cheiroarthropathy[20][21] +

(waxy skin)

- - - + - +
  • N/A
  • N/A
  • N/A
Myxedema[22] +

(coarse skin)

- - + - - -
  • Anti-TPO antibody
  • Anti-Tg antibody
  • N/A
 Serum TSH
Renal diseases Nephrogenic systemic fibrosis[23] +

(induration)

- - + + - -
  • N/A
  • N/A

References

  1. ↑ LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA, Rowell N, Wollheim F (February 1988). "Scleroderma (systemic sclerosis): classification, subsets and pathogenesis". J. Rheumatol. 15 (2): 202–5. PMID 3361530.
  2. ↑ Black CM (August 1993). "Scleroderma--clinical aspects". J. Intern. Med. 234 (2): 115–8. PMID 8340733.
  3. ↑ Cutolo M, Sulli A, Smith V (April 2013). "How to perform and interpret capillaroscopy". Best Pract Res Clin Rheumatol. 27 (2): 237–48. doi:10.1016/j.berh.2013.03.001. PMID 23731933.
  4. ↑ 4.0 4.1 van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA, Carreira PE, Riemekasten G, Clements PJ, Denton CP, Distler O, Allanore Y, Furst DE, Gabrielli A, Mayes MD, van Laar JM, Seibold JR, Czirjak L, Steen VD, Inanc M, Kowal-Bielecka O, Müller-Ladner U, Valentini G, Veale DJ, Vonk MC, Walker UA, Chung L, Collier DH, Ellen Csuka M, Fessler BJ, Guiducci S, Herrick A, Hsu VM, Jimenez S, Kahaleh B, Merkel PA, Sierakowski S, Silver RM, Simms RW, Varga J, Pope JE (November 2013). "2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative". Ann. Rheum. Dis. 72 (11): 1747–55. doi:10.1136/annrheumdis-2013-204424. PMID 24092682.
  5. ↑ Cutolo M, Sulli A, Smith V (April 2013). "How to perform and interpret capillaroscopy". Best Pract Res Clin Rheumatol. 27 (2): 237–48. doi:10.1016/j.berh.2013.03.001. PMID 23731933.
  6. ↑ Rongioletti F, Kaiser F, Cinotti E, Metze D, Battistella M, Calzavara-Pinton PG, Damevska K, Girolomoni G, André J, Perrot JL, Kempf W, Cavelier-Balloy B (December 2015). "Scleredema. A multicentre study of characteristics, comorbidities, course and therapy in 44 patients". J Eur Acad Dermatol Venereol. 29 (12): 2399–404. doi:10.1111/jdv.13272. PMID 26304054.
  7. ↑ Rongioletti F, Rebora A (February 2001). "Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema". J. Am. Acad. Dermatol. 44 (2): 273–81. doi:10.1067/mjd.2001.111630. PMID 11174386.
  8. ↑ Gabriel SE, Perry HO, Oleson GB, Bowles CA (January 1988). "Scleromyxedema: a scleroderma-like disorder with systemic manifestations". Medicine (Baltimore). 67 (1): 58–65. PMID 3336281.
  9. ↑ Herson S, Brechignac S, Piette JC, Mouthon JM, Coutellier A, Bletry O, Godeau P (June 1990). "Capillary microscopy during eosinophilic fasciitis in 15 patients: distinction from systemic scleroderma". Am. J. Med. 88 (6): 598–600. PMID 2346160.
  10. ↑ Falanga V, Medsger TA (October 1987). "Frequency, levels, and significance of blood eosinophilia in systemic sclerosis, localized scleroderma, and eosinophilic fasciitis". J. Am. Acad. Dermatol. 17 (4): 648–56. PMID 3668010.
  11. ↑ Schaffer JV, McNiff JM, Seropian S, Cooper DL, Bolognia JL (October 2005). "Lichen sclerosus and eosinophilic fasciitis as manifestations of chronic graft-versus-host disease: expanding the sclerodermoid spectrum". J. Am. Acad. Dermatol. 53 (4): 591–601. doi:10.1016/j.jaad.2005.06.015. PMID 16198778.
  12. ↑ Martires KJ, Baird K, Steinberg SM, Grkovic L, Joe GO, Williams KM, Mitchell SA, Datiles M, Hakim FT, Pavletic SZ, Cowen EW (October 2011). "Sclerotic-type chronic GVHD of the skin: clinical risk factors, laboratory markers, and burden of disease". Blood. 118 (15): 4250–7. doi:10.1182/blood-2011-04-350249. PMC 3204741. PMID 21791415.
  13. ↑ Finch WR, Rodnan GP, Buckingham RB, Prince RK, Winkelstein A (1980). "Bleomycin-induced scleroderma". J. Rheumatol. 7 (5): 651–9. PMID 6160247.
  14. ↑ Passiu G, Cauli A, Atzeni F, Aledda M, Dessole G, Sanna G, Nurchis P, Vacca A, Garau P, Laudadio M, Mathieu A (1999). "Bleomycin-induced scleroderma: report of a case with a chronic course rather than the typical acute/subacute self-limiting form". Clin. Rheumatol. 18 (5): 422–4. PMID 10524560.
  15. ↑ Satoh M, Chan EK, Sobel ES, Kimpel DL, Yamasaki Y, Narain S, Mansoor R, Reeves WH (September 2007). "Clinical implication of autoantibodies in patients with systemic rheumatic diseases". Expert Rev Clin Immunol. 3 (5): 721–38. doi:10.1586/1744666X.3.5.721. PMID 20477023.
  16. ↑ Moinzadeh P, Aberer E, Ahmadi-Simab K, Blank N, Distler JH, Fierlbeck G, Genth E, Guenther C, Hein R, Henes J, Herich L, Herrgott I, Koetter I, Kreuter A, Krieg T, Kuhr K, Lorenz HM, Meier F, Melchers I, Mensing H, Mueller-Ladner U, Pfeiffer C, Riemekasten G, Sárdy M, Schmalzing M, Sunderkoetter C, Susok L, Tarner IH, Vaith P, Worm M, Wozel G, Zeidler G, Hunzelmann N (April 2015). "Disease progression in systemic sclerosis-overlap syndrome is significantly different from limited and diffuse cutaneous systemic sclerosis". Ann. Rheum. Dis. 74 (4): 730–7. doi:10.1136/annrheumdis-2013-204487. PMC 4392314. PMID 24389298.
  17. ↑ Foocharoen C, Netwijitpan S, Mahakkanukrauh A, Suwannaroj S, Nanagara R (September 2016). "Clinical characteristics of scleroderma overlap syndromes: comparisons with pure scleroderma". Int J Rheum Dis. 19 (9): 913–23. doi:10.1111/1756-185X.12884. PMID 27126733.
  18. ↑ Pakozdi A, Nihtyanova S, Moinzadeh P, Ong VH, Black CM, Denton CP (November 2011). "Clinical and serological hallmarks of systemic sclerosis overlap syndromes". J. Rheumatol. 38 (11): 2406–9. doi:10.3899/jrheum.101248. PMID 21844148.
  19. ↑ Aletaha D, Neogi T, Silman AJ, Funovits J, Felson DT, Bingham CO, Birnbaum NS, Burmester GR, Bykerk VP, Cohen MD, Combe B, Costenbader KH, Dougados M, Emery P, Ferraccioli G, Hazes JM, Hobbs K, Huizinga TW, Kavanaugh A, Kay J, Kvien TK, Laing T, Mease P, Ménard HA, Moreland LW, Naden RL, Pincus T, Smolen JS, Stanislawska-Biernat E, Symmons D, Tak PP, Upchurch KS, Vencovský J, Wolfe F, Hawker G (September 2010). "2010 Rheumatoid arthritis classification criteria: an American College of Rheumatology/European League Against Rheumatism collaborative initiative". Arthritis Rheum. 62 (9): 2569–81. doi:10.1002/art.27584. PMID 20872595.
  20. ↑ Seibold JR (November 1982). "Digital sclerosis in children with insulin-dependent diabetes mellitus". Arthritis Rheum. 25 (11): 1357–61. PMID 6753855.
  21. ↑ Jelinek JE (April 1993). "The skin in diabetes". Diabet. Med. 10 (3): 201–13. PMID 8485952.
  22. ↑ Heymann WR (June 1992). "Cutaneous manifestations of thyroid disease". J. Am. Acad. Dermatol. 26 (6): 885–902. PMID 1607406.
  23. ↑ Galan A, Cowper SE, Bucala R (November 2006). "Nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy)". Curr Opin Rheumatol. 18 (6): 614–7. doi:10.1097/01.bor.0000245725.94887.8d. PMID 17053507.

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