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].

Differentiating Scleroderma from other Diseases

		Symptoms			Physical examination				Lab Findings		Imaging	Histopathology
Diseases		Clinical manifestations							Para-clinical findings				Gold standard	Additional findings
		Symptoms			Physical examination				Para-clinical findings
		Skin thickening	Raynaud phenomenon	Heart burn	Edema (swelling)	Sclerodactyly	Telangiectasia	Impaired mobility	Autoantibodies	Blood indices	Imaging	Histopathology
Scleroderma^[1]^[2]	Limited cutaneous systemic sclerosis (CREST syndrome)	+ (induration)	+	+	+/-	+	+	+/-	Anti-centromere antibody Antinuclear antibody (ANA)	Anemia Elevated creatinine level	Nailfold microvascular changes Chest CT showing evidence of pulmonary fibrosis	Nailfold microvascular changes Dilated capillary loops Microhemmorhages		Interstitial lung disease Pulmonary hypertension Calcinosis Dyspnea
Scleroderma^[1]^[2]	Diffuse cutaneous systemic sclerosis	+ (induration)	+	+	+/-	+	+	+/-	Anti-topoisomerase-I (Scl-70) antibody Anti-RNA polymerase III antibody Antinuclear antibody (ANA)	Anemia Elevated creatinine level	Nailfold microvascular changes	Nailfold microvascular changes Dilated capillary loops Microhemmorhages		Interstitial lung disease Pulmonary hypertension Scleroderma renal crisis
Systemic diseases	Scleredema (Buschke's disease)^[3]	+	-	-	+	-	-	+	-	Fasting blood sugar HbA1C	-	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)^[4]^[5]	+ (waxy yellow-red papules)	+/-	+/-	+	-	-	-	-	Monoclonal gammopathy (IgG-lambda) Normal TSH	-	Fibroblast proliferation in the dermis Mucin deposition in the dermis		Associated with malignancies AL amyloidosis Normal thyroid function
	Eosinophilic fasciitis^[6]^[7]	+ (orange peel-peau d'orange appearance)	-	-	+	-	-	-	-	Eosinophilia (peripheral blood smear)	Normal appearance on nailfold video capillaroscopy (NVC)	Fascia is edematous and infiltrated by eosinophils		Fasciitis present on the trunk sparing extremities Visible collapse of superficial veins when the limb is elevated
	Chronic graft-versus-host disease^[8]	+ (induration)	-	-		-	-	-	Antinuclear antibody (ANA) Anti-mitochondrial antibody					Occurs after allogeneic hematopoietic transplantation
	Drug induced scleroderma^[9]	+	+	+/-	+/-	+	+/-	+/-	-	-	-			Bleomycin
Scleroderma overlap syndromes^[10]^[11]^[12]^[13]	Systemic lupus erythematosus	+ (rash)	+	+	+/-	+	+	+/-	Anti-Ro52 Antinuclear antibody (ANA) Anti-Smith antibody Anti-dsDNA antibody Antiphospholipid antibody Anti-centromere antibody (ACA) Anti-topoisomerase-I (Scl-70) antibody Anti-RNA polymerase III antibody	Decreased C3, C4 and CH50 Anemia		Inflammation at the dermal-epidermal junction		Malar (butterfly) rash Arthritis
	Polymyositis	+ (rash)	+	+	+/-	+	+	+/-	Anti-Ro52 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	Elevated creatinine kinase Aldolase Anemia		Inflammation at the dermal-epidermal junction Atrophy of the epidermis Perivascular infiltrate in the dermis		Symmetric proximal muscle weakness
	Rheumatoid arthritis	+ (rash)	+	+	+/-	+	+	+/-	Anti-Ro52 Antinuclear antibody (ANA) Antinuclear antibody (ANA) Rheumatoid factor (RA) +ve Anti-CCP antibody Anti-centromere antibody (ACA) Anti-topoisomerase-I (Scl-70) antibody Anti-RNA polymerase III antibody	Elevated ESR Anemia		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)^[14]^[15]	+ (waxy skin)	-	-	-	+	-	+	-			-		Long standing type I diabetes
Endocrine disorders	Myxedema due to hypothyroidism^[16]	+ (coarse skin)	-	-	+	-	-	-	Anti-TPO antibody Anti-Tg antibody	Anemia Serum TSH		Atrophied epidermis Hyperkeratosis		Hypothyroidism
Renal diseases	Nephrogenic systemic fibrosis^[17]	+ (induration)	-	-	+	+	-	-	-	Elevated C-reactive protein Elevated ESR Decreased serum albumin Decreased creatinine clearance		Expansion and fibrosis of dermis Accumulation of CD34 positive fibroblasts		Skin changes on extremities and trunk Affects patients with advanced deals dependent renal failure Gadolinium containing contrast exposure is a risk factor

References

↑ 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.
↑ Black CM (August 1993). "Scleroderma--clinical aspects". J. Intern. Med. 234 (2): 115–8. PMID 8340733.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ Finch WR, Rodnan GP, Buckingham RB, Prince RK, Winkelstein A (1980). "Bleomycin-induced scleroderma". J. Rheumatol. 7 (5): 651–9. PMID 6160247.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ Seibold JR (November 1982). "Digital sclerosis in children with insulin-dependent diabetes mellitus". Arthritis Rheum. 25 (11): 1357–61. PMID 6753855.
↑ Jelinek JE (April 1993). "The skin in diabetes". Diabet. Med. 10 (3): 201–13. PMID 8485952.
↑ Heymann WR (June 1992). "Cutaneous manifestations of thyroid disease". J. Am. Acad. Dermatol. 26 (6): 885–902. PMID 1607406.
↑ 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.

Template:WH Template:WS

[pmid3361530-1] 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.

[pmid8340733-2] Black CM (August 1993). "Scleroderma--clinical aspects". J. Intern. Med. 234 (2): 115–8. PMID 8340733.

[pmid26304054-3] 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.

[pmid11174386-4] 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.

[pmid3336281-5] 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.

[pmid2346160-6] 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.

[pmid3668010-7] 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.

[pmid16198778-8] 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.

[pmid6160247-9] Finch WR, Rodnan GP, Buckingham RB, Prince RK, Winkelstein A (1980). "Bleomycin-induced scleroderma". J. Rheumatol. 7 (5): 651–9. PMID 6160247.

[pmid20477023-10] 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.

[pmid24389298-11] 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.

[pmid27126733-12] 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.

[pmid21844148-13] 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.

[pmid6753855-14] Seibold JR (November 1982). "Digital sclerosis in children with insulin-dependent diabetes mellitus". Arthritis Rheum. 25 (11): 1357–61. PMID 6753855.

[pmid8485952-15] Jelinek JE (April 1993). "The skin in diabetes". Diabet. Med. 10 (3): 201–13. PMID 8485952.

[pmid1607406-16] Heymann WR (June 1992). "Cutaneous manifestations of thyroid disease". J. Am. Acad. Dermatol. 26 (6): 885–902. PMID 1607406.

[pmid17053507-17] 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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Differentiating Scleroderma from other diseases

Overview

Differentiating X from other Diseases

Differentiating Scleroderma from other Diseases

References

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