Differentiating Scleroderma from other diseases: Difference between revisions

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|- 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="8" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Clinical manifestations'''
| colspan="10" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Clinical manifestations'''
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! colspan="7" 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'''
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| colspan="4" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Symptoms'''
| colspan="4" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Symptoms'''
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! colspan="4" 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
!
!
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|-
!
!
!
!
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab Findings
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab Findings
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging
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! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Dysphagia
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Dysphagia
!Dyspnea
!Dyspnea
!Impaired mobility
!Swelling
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Indurated skin
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Indurated skin
! 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
!Abdominal distension
!Abdominal distension
!Impaired mobility
!Edema
! 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;" |Anemia
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Anemia
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab 3
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Peripheral blood smear
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging 1
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Nailfold video capillaroscopy
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging 2
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |CT-scan
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging 3
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging 3
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| 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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| style="background: #F5F5F5; padding: 5px;" |+
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|<nowiki>+/-</nowiki>
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| +/-
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|<nowiki>+/-</nowiki>
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| style="background: #F5F5F5; padding: 5px;" |Anti-centromere antibody
ANA
| style="background: #F5F5F5; padding: 5px;" |+/-
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |Nailfold microvascular changes
| style="background: #F5F5F5; padding: 5px;" |Chest CT showing evidence of pulmonary fibrosis
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |Nailfold microvascular changes
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |Interstitial lung disease / Pulmonary hypertension
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| 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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|<nowiki>+/-</nowiki>
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| +/-
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| +/-
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|<nowiki>+/-</nowiki>
| +/-
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| style="background: #F5F5F5; padding: 5px;" |Anti-Scl-70 antibody
ANA
| 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;" |Nailfold microvascular changes
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| rowspan="6" style="background: #DCDCDC; padding: 5px; text-align: center;" |Systemic diseases
| rowspan="6" style="background: #DCDCDC; padding: 5px; text-align: center;" |Systemic diseases
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Scleroderma
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Scleredema
| style="background: #F5F5F5; padding: 5px;" |
(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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* Normal epidermis
* Thickened dermis with swelling of collagen
* Fibroblast proliferation is absent in the dermis
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* symmetrical skin thickening
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* trunk, shoulders, upper back, face
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* fingers are spared
* Mobility of shoulders and chest
* Internal organ involvement rare
* Associated with viral URTI and Diabetes mellitus type 1, monoclonal gammopathy
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| style="background: #DCDCDC; padding: 5px; text-align: center;" |Scleromyxedema
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Scleromyxedema
(lichen myxedematosus)
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| style="background: #F5F5F5; padding: 5px;" |Fibroblast proliferation in the dermis
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| style="background: #F5F5F5; padding: 5px;" |Eosinophilia
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Revision as of 18:51, 19 April 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

[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].

OR

[Disease name] must be differentiated from [[differential dx1], [differential dx2], and [differential dx3].

Differentiating X from other Diseases

  • [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].
  • [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

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging Histopathology
Skin thickening Raynaud phenomenon Dysphagia Dyspnea Impaired mobility Swelling Indurated skin Sclerodactyly Telangiectasia Abdominal distension Impaired mobility Edema Autoantibodies Anemia Peripheral blood smear Nailfold video capillaroscopy CT-scan Imaging 3
Scleroderma Limited cutaneous systemic sclerosis (CREST syndrome) + + +/- +/- + + + +/- Anti-centromere antibody

ANA

+/- Nailfold microvascular changes Chest CT showing evidence of pulmonary fibrosis Nailfold microvascular changes Interstitial lung disease / Pulmonary hypertension
Diffuse cutaneous systemic sclerosis + + +/- +/- +/- + + + +/- +/- Anti-Scl-70 antibody

ANA

+/- Nailfold microvascular changes
Systemic diseases Scleredema

(Buschke's disease)[1]

+ - - - + + - - - + - - -
  • Normal epidermis
  • Thickened dermis with swelling of collagen
  • Fibroblast proliferation is absent in the dermis
  • symmetrical skin thickening
  • trunk, shoulders, upper back, face
  • fingers are spared
  • Mobility of shoulders and chest
  • Internal organ involvement rare
  • Associated with viral URTI and Diabetes mellitus type 1, monoclonal gammopathy
Scleromyxedema

(lichen myxedematosus)

Fibroblast proliferation in the dermis
Amyloidosis
Eosinophilic fasciitis + Eosinophilia
Chronic graft-versus-host disease
Drug induced scleroderma
Scleroderma overlap syndromes Systemic lupus erythematosus
Dermatomyositis
Rheumatoid arthritis
Endocrine disorders Diabetes mellitus (diabetic cheiroarthropathy)
Myxedema due to hypothyroidism
POEMS syndrome
Renal diseases Nephrogenic systemic fibrosis

References

  1. 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.

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