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)	Anemia		Nailfold microvascular changes
Systemic diseases	Scleredema (Buschke's disease)	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	Systemic lupus erythematosus	Skin thickening/ rash	+	+	+/-	+/-	+	+	+/-
	Dermatomyositis	Skin thickening/ rash	+	+	+/-	+/-	+	+	+/-
	Rheumatoid arthritis	Skin thickening/ rash	+	+	+/-	+/-	+	+	+/-
Endocrine disorders	Diabetes mellitus (diabetic cheiroarthropathy)
	Myxedema due to hypothyroidism
	POEMS syndrome
Renal diseases	Nephrogenic systemic fibrosis