Differentiating Scleroderma from other diseases: Difference between revisions

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__NOTOC__
__NOTOC__
{{Scleroderma}}
[[Image:Home_logo1.png|right|250px|link=https://www.wikidoc.org/index.php/Scleroderma]]
{{CMG}}; {{AE}} {{MKA}}
{{CMG}}; {{AE}} {{MKA}}


==Overview==
==Overview==
[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as [differential dx1], [differential dx2], and [differential dx3].
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]].


OR
==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]].


[Disease name] must be differentiated from [[differential dx1], [differential dx2], and [differential dx3].
===Differentiating Scleroderma from other Diseases===


==Differentiating X from other Diseases==
<small><small>
*[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as [differential dx1], [differential dx2], and [differential dx3].
<div style="width: 60%;">
*[Disease name] must be differentiated from [differential dx1], [differential dx2], and [differential dx3].
 
*As [disease name] manifests in a variety of clinical forms, differentiation must be established in accordance with the particular subtype. [Subtype name 1] must be differentiated from other diseases that cause [clinical feature 1], such as [differential dx1] and [differential dx2]. In contrast, [subtype name 2] must be differentiated from other diseases that cause [clinical feature 2], such as [differential dx3] and [differential dx4].
 
===Preferred Table===
{|
{|
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
! colspan="2" rowspan="4" |Diseases
! colspan="2" rowspan="4" |Diseases
| colspan="7" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Clinical manifestations'''
| colspan="7" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Clinical manifestations'''
|
! colspan="4" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Para-clinical findings
! colspan="7" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Para-clinical findings
| colspan="1" rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Gold standard'''
| colspan="1" rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Gold standard'''
! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Additional findings
! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Additional findings
|-
|-
| colspan="4" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Symptoms'''
| colspan="3" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Symptoms'''
! colspan="3" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical examination
! colspan="4" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical examination
!
|-
|-
!
! colspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab Findings
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab Findings
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histopathology
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histopathology
|-  
|-  
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Skin changes
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Skin thickening
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Raynaud phenomenon
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Raynaud phenomenon
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Dysphagia
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Heart burn
!Dyspnea
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Edema (swelling)
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Edema (swelling)
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Sclerodactyly
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Sclerodactyly
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Telangiectasia
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Telangiectasia
!Impaired mobility
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Impaired mobility
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Autoantibodies
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Autoantibodies
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Blood indices
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Blood indices
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Nailfold video capillaroscopy
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |CT-scan
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging 3
|-
|-
| rowspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Scleroderma
| 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]])'''
| style="background: #F5F5F5; padding: 5px;" |Skin thickening (induration)
| style="background: #F5F5F5; padding: 5px;" | +
([[induration]])
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
|<nowiki>+/-</nowiki>
| 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;" | +
|
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Anti-centromere antibody
* [[Anti-centromere antibodies|Anti-centromere antibody]]
* Antinuclear antibody (ANA)
* [[Antinuclear antibody|Antinuclear antibody (ANA)]]
| style="background: #F5F5F5; padding: 5px;" |Anemia
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |Nailfold microvascular changes
* [[Anemia]]
| style="background: #F5F5F5; padding: 5px;" |Chest CT showing evidence of pulmonary fibrosis
* Elevated [[creatinine]] level
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |Nailfold microvascular changes
* Nailfold microvascular changes on nailfold video capillaroscopy (NVC)<ref name="pmid23731933">{{cite journal |vauthors=Cutolo M, Sulli A, Smith V |title=How to perform and interpret capillaroscopy |journal=Best Pract Res Clin Rheumatol |volume=27 |issue=2 |pages=237–48 |date=April 2013 |pmid=23731933 |doi=10.1016/j.berh.2013.03.001 |url=}}</ref>
* Chest [[CT|HRCT]] scan showing evidence of [[pulmonary fibrosis]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |Interstitial lung disease / Pulmonary hypertension
* Nailfold microvascular changes
* Dilated [[capillary]] loops
* Microhemmorhages
| style="background: #F5F5F5; padding: 5px;" |
* [[American College of Rheumatology|American College of Rheumatology (ACR)]]/ European League Against Rheumatism (EULAR) classification criteria<ref name="pmid24092682">{{cite journal |vauthors=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 |title=2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative |journal=Ann. Rheum. Dis. |volume=72 |issue=11 |pages=1747–55 |date=November 2013 |pmid=24092682 |doi=10.1136/annrheumdis-2013-204424 |url=}}</ref>
| style="background: #F5F5F5; padding: 5px;" |
* [[Interstitial lung disease]]
* [[Pulmonary hypertension]]
* [[Calcinosis]]
* [[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]]'''
| style="background: #F5F5F5; padding: 5px;" |Skin thickening (induration)
| style="background: #F5F5F5; padding: 5px;" | +
([[induration]])
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
|<nowiki>+/-</nowiki>
| 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;" | +
| +/-
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Anti-Scl-70 antibody
* Anti-topoisomerase-I (Scl-70) antibody
* Antinuclear antibody (ANA)
* Anti-RNA polymerase III antibody
* Anti-RNA polymerase III antibody
| style="background: #F5F5F5; padding: 5px;" |Anemia
* [[Antinuclear antibody|Antinuclear antibody (ANA)]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |Nailfold microvascular changes
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Anemia]]
* Elevated [[creatinine]] level
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Nailfold microvascular changes on nailfold video capillaroscopy (NVC)<ref name="pmid237319332">{{cite journal |vauthors=Cutolo M, Sulli A, Smith V |title=How to perform and interpret capillaroscopy |journal=Best Pract Res Clin Rheumatol |volume=27 |issue=2 |pages=237–48 |date=April 2013 |pmid=23731933 |doi=10.1016/j.berh.2013.03.001 |url=}}</ref>
* Chest [[CT|HRCT]] scan showing evidence of [[pulmonary fibrosis]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Nailfold microvascular changes
* Dilated [[capillary]] loops
* Microhemmorhages
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[American College of Rheumatology|American College of Rheumatology (ACR)]]/ European League Against Rheumatism (EULAR) classification criteria<ref name="pmid24092682" />
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Interstitial lung disease]]
* [[Pulmonary hypertension]]
* Scleroderma [[renal]] crisis
* [[Dyspnea]]
|-
|-
| rowspan="6" 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>'''
| style="background: #F5F5F5; padding: 5px;" |Skin thickening
| 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;" | -
|<nowiki>-</nowiki>
| 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;" | -
| +
| 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]]
* [[HbA1C]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* N/A
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Normal epidermis
* Normal [[epidermis]]
* Thickened dermis with swelling of collagen
* Thickened [[dermis]] with swelling of [[collagen]]
* Fibroblast proliferation is absent in the dermis
* [[Fibroblast]] proliferation is absent in the [[dermis]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Skin biopsy]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* symmetrical skin thickening
* Symmetrical [[skin]] thickening
* trunk, shoulders, upper back, face
* Trunk, shoulders, upper back, face
* fingers are spared
* Fingers are spared
* Mobility of shoulders and chest
* Immobility of shoulders and chest
* Internal organ involvement rare
* Associated with [[URTI|viral URTI]] and [[diabetes mellitus type 1]], [[monoclonal gammopathy]]
* Associated with 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)
'''([[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>
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |
 
| style="background: #F5F5F5; padding: 5px;" |
(waxy yellow-red [[papules]])
|
| 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;" | -
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* N/A
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Monoclonal gammopathy]] (IgG-lambda)
* Normal [[TSH]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
|
* N/A
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Fibroblast]] proliferation in the [[dermis]]
* [[Mucin]] deposition in the [[dermis]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Skin biopsy]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Associated with [[malignancies]]
| style="background: #F5F5F5; padding: 5px;" |
* [[AL amyloidosis]]
| style="background: #F5F5F5; padding: 5px;" |
* Normal [[thyroid]] function
| style="background: #F5F5F5; padding: 5px;" |Fibroblast proliferation in the dermis
* [[Scalp]] and head are affected
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Amyloidosis
| 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>
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |
 
| style="background: #F5F5F5; padding: 5px;" |
(orange peel-[[peau d'orange]] appearance)
|
| 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;" |
| 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;" |
| 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]])
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Normal appearance on nailfold video capillaroscopy (NVC)
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Fascia]] is edematous and infiltrated by [[eosinophils]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Skin biopsy]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Fasciitis]] present on the trunk sparing extremities
* Visible collapse of [[superficial veins]] when the limb is elevated
* Hands and feet are not involved
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Eosinophilic fasciitis
| 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;" | +
| style="background: #F5F5F5; padding: 5px;" |
(induration)
| 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;" | -
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Antinuclear antibody|Antinuclear antibody (ANA)]]
* [[Anti-mitochondrial antibody]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
|
* Increased [[serum bilirubin]] concentration
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |Eosinophilia (peripheral blood smear)
| 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;" |
* Thick [[collagen]] bundles in the [[dermis]]
* Normal appearing [[epidermis]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Skin biopsy]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Occurs after [[allogeneic]] [[hematopoietic]] [[transplantation]]
* [[Pulmonary fibrosis]]
* [[Esophageal]] sclerosis
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Chronic graft-versus-host disease
| 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: #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;" | +/-
|
| 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;" |
| 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;" |
* N/A
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* N/A
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[IgG]] and [[complement]] deposits on [[epidermal]] [[keratinocytes]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Skin biopsy]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Bleomycin]]
* Cancer [[chemotherapeutic]] drugs
* Skin changes present on trunk and extremities
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Drug induced scleroderma
| 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: #F5F5F5; padding: 5px;" |
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Scleroderma-[[systemic lupus erythematosus]] overlap'''
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |
(rash)
|
| 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;" |
| 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;" |
|-
| 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>
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Systemic lupus erythematosus
| style="background: #F5F5F5; padding: 5px;" |Skin thickening/ rash
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
|<nowiki>+/-</nowiki>
| 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;" | +
|<nowiki>+/-</nowiki>
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* '''Anti-Ro52 antibody'''
* [[Antinuclear antibody|Antinuclear antibody (ANA)]]
* Anti-Smith antibody
* [[Anti-dsDNA antibody]]
* [[Antiphospholipid antibody]]
* Anti-centromere antibody (ACA)
* Anti-centromere antibody (ACA)
* Anti-topoisomerase-I (Scl-70) antibody
* Anti-topoisomerase-I (Scl-70) antibody
* Anti-RNA polymerase III antibody
* Anti-RNA polymerase III antibody
* Antinuclear antibody (ANA)
* Anti-Sm antibody
* Anti-dsDNA antibody
* Antiphospholipid antibody
* Anti-Ro52
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Decreased [[C3 (complement)|C3]], C4 and CH50
* [[Anemia]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* N/A
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |Inflammation at the dermal-epidermal junction
* [[Inflammation]] at the [[dermal]]-[[epidermal]] junction
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Anti-Ro52 antibody
* [[Anti-dsDNA antibody]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Malar]] (butterfly) [[rash]]
* [[Arthritis]]
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Dermatomyositis
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Scleroderma-[[polymyositis]] overlap'''
| style="background: #F5F5F5; padding: 5px;" |Skin thickening/ rash
| style="background: #F5F5F5; padding: 5px;" | +
(rash)
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
|<nowiki>+/-</nowiki>
| 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;" | +
|<nowiki>+/-</nowiki>
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* '''Anti-Ro52 antibody'''
* [[Antinuclear antibody|Antinuclear antibody (ANA)]]
* Anti-Jo-1 antibody
* Anti-SRP antibody
* Anti-Mi-2 antibody
* Anti-centromere antibody (ACA)
* Anti-centromere antibody (ACA)
* Anti-topoisomerase-I (Scl-70) antibody
* Anti-topoisomerase-I (Scl-70) antibody
* Anti-RNA polymerase III antibody
* Anti-RNA polymerase III antibody
* Antinuclear antibody (ANA)
* Anti-Ro52
| style="background: #F5F5F5; padding: 5px;" |Elevated creatinine kinase
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Elevated [[creatine kinase]]
* [[Aldolase]]
* [[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
* [[Atrophy]] of the [[epidermis]]
* Perivascular infiltrate in the [[dermis]]
* [[Necrosis]] and [[atrophy]] of endomysial [[connective tissue]] on [[muscle biopsy]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |Inflammation at the dermal-epidermal junction
* Anti-Ro52 antibody
* [[Muscle biopsy]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |Proximal muscle weakness
* Symmetric [[proximal muscle weakness]]
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Rheumatoid arthritis
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Scleroderma-[[rheumatoid arthritis]] overlap'''
| style="background: #F5F5F5; padding: 5px;" |Skin thickening/ rash
| style="background: #F5F5F5; padding: 5px;" | +
(rash)
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
|<nowiki>+/-</nowiki>
| 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;" | +
|<nowiki>+/-</nowiki>
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* '''Anti-Ro52 antibody'''
* [[Antinuclear antibody|Antinuclear antibody (ANA)]]
* [[Rheumatoid factor]] (RF) +ve
* Anti-CCP antibody
* Anti-centromere antibody (ACA)
* Anti-centromere antibody (ACA)
* Anti-topoisomerase-I (Scl-70) antibody
* Anti-topoisomerase-I (Scl-70) antibody
* Anti-RNA polymerase III antibody
* Anti-RNA polymerase III antibody
* Antinuclear antibody (ANA)
* Antinuclear antibody (ANA)
* Rheumatoid factor (RA) +ve
* Anti-CCP antibody
* Anti-Ro52
| style="background: #F5F5F5; padding: 5px;" |Elevated ESR
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Elevated [[ESR]]
* [[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
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |Inflammation at the dermal-epidermal junction
* Anti-Ro52 antibody
* Anti-CCP antibody
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |Clinical findings that suggest rheumatoid arthritis (RA) but do not fulfill the ACR criteria for RA
* 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="3" 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;" |Diabetes mellitus (diabetic cheiroarthropathy)
| 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;" | +
| style="background: #F5F5F5; padding: 5px;" |
 
| style="background: #F5F5F5; padding: 5px;" |
(waxy skin)
|
| 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;" |
| 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;" |
| 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
* [[HbA1C]] ≥ 6.5%
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* N/A
| 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
* [[HbA1C]] ≥ 6.5%
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Long standing [[type I diabetes mellitus]]
* [[Skin]] thickening of fingers
* Flexion [[contractures]] on fingers
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Myxedema due to hypothyroidism
| 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;" | +
| style="background: #F5F5F5; padding: 5px;" |
 
| style="background: #F5F5F5; padding: 5px;" |
(coarse skin)
|
| 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;" |
| 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;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Anti-TPO antibody
* Anti-Tg antibody
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Anemia]]
* 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]]
* [[Hyperkeratosis]]
| style="background: #F5F5F5; padding: 5px;" |Serum [[TSH]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Hypothyroidism]]
* Nonpitting [[edema]] present
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |POEMS syndrome
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Renal diseases'''
| style="background: #F5F5F5; padding: 5px;" |
| 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;" | +
| style="background: #F5F5F5; padding: 5px;" |
(induration)
|
| 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;" |
| 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;" |
| 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;" |
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Renal diseases
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Nephrogenic systemic fibrosis
| 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;" |
|
| 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 [[ESR]]
* Decreased [[serum albumin]]
* 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]]
* Accumulation of [[CD34]] positive [[fibroblasts]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Skin biopsy]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Skin]] changes on [[extremities]] and [[trunk]] (involves hand and feet)
* Affects patients with advanced [[dialysis]] dependent [[renal failure]]
* [[Gadolinium]] containing [[contrast]] exposure is a risk factor
* Internal organ [[fibrosis]] can occur
|}
|}
</small></small>
</div>


==References==
==References==
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{{WH}}
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[[Category: (name of the system)]]
 
[[Category:Up-To-Date]]
[[Category:Medicine]]
[[Category:Dermatology]]
[[Category:Rheumatology]]

Latest revision as of 21:23, 29 July 2020

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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