Differentiating Scleroderma from other diseases

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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 changes Raynaud phenomenon Dysphagia Dyspnea Edema (swelling) Sclerodactyly Telangiectasia Impaired mobility Autoantibodies Blood indices Nailfold video capillaroscopy CT-scan Imaging 3
Scleroderma Limited cutaneous systemic sclerosis (CREST syndrome) Skin thickening (induration) + + +/- +/- + +
  • Anti-centromere antibody
  • Antinuclear antibody (ANA)
Anemia Nailfold microvascular changes Chest CT showing evidence of pulmonary fibrosis Nailfold microvascular changes Interstitial lung disease / Pulmonary hypertension
Diffuse cutaneous systemic sclerosis Skin thickening (induration) + + +/- +/- + + +/-
  • Anti-Scl-70 antibody
  • Antinuclear antibody (ANA)
  • Anti-RNA polymerase III antibody
Anemia Nailfold microvascular changes
Systemic diseases Scleredema

(Buschke's disease)[1]

Skin thickening - - - + - - + - - -
  • 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 (peripheral blood smear)
Chronic graft-versus-host disease
Drug induced scleroderma
Scleroderma overlap syndromes[2][3][4] Systemic lupus erythematosus Skin thickening/ rash + + +/- +/- + + +/-
  • Anti-centromere antibody (ACA)
  • Anti-topoisomerase-I (Scl-70) antibody
  • Anti-RNA polymerase III antibody
  • Antinuclear antibody (ANA)
  • Anti-Sm antibody
  • Anti-dsDNA antibody
  • Antiphospholipid antibody
  • Anti-Ro52
Inflammation at the dermal-epidermal junction
Polymyositis Skin thickening/ rash + + +/- +/- + + +/-
  • Anti-centromere antibody (ACA)
  • Anti-topoisomerase-I (Scl-70) antibody
  • Anti-RNA polymerase III antibody
  • Antinuclear antibody (ANA)
  • Anti-Jo-1 antibody
  • Anti-SRP antibody
  • Anti-Mi-2 antibody
  • Anti-Ro52
  • Elevated creatinine kinase
  • Aldolase
  • Inflammation at the dermal-epidermal junction
  • Atrophy of the epidermis
  • Perivascular infiltrate in the dermis
Symmetric proximal muscle weakness
Rheumatoid arthritis Skin thickening/ rash + + +/- +/- + + +/-
  • Anti-centromere antibody (ACA)
  • Anti-topoisomerase-I (Scl-70) antibody
  • Anti-RNA polymerase III antibody
  • Antinuclear antibody (ANA)
  • Antinuclear antibody (ANA)
  • Rheumatoid factor (RA) +ve
  • Anti-CCP antibody
  • Anti-Ro52
Elevated ESR Inflammation at the dermal-epidermal junction Clinical findings that suggest rheumatoid arthritis (RA) but do not fulfill the ACR criteria for RA
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.
  2. 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.
  3. 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.
  4. 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.

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