Table 2.

Differentiating features between scleroderma and scleroderma-like fibrosing disorders.

	Scleroderma	Scleromyxedema	Scleredema	Eosinophilic Fasciitis	Nephrogenic Systemic Fibrosis
Skin findings
Quality	Indurated, thick	Papular, waxy	Indurated, doughy	Woody induration	Cobblestone, nodular, indurated plaques
Distribution	Fingers, hands, extremities, face, chest. Back spared	Face, neck, extremities, fingers	Neck, back, face	Extremities, trunk. Hands and feet spared	Extremities, trunk, hand, feet. Face spared
Systemic Disease
Raynaud’s phenomenon	Almost universal	Not common	No	Unusual	Unusual
Nailfold capillaries	Universally abnormal	Normal	Normal	Normal	Normal
Antinuclear Antibody	Positive 95–100%	Uncommon	Negative	Uncommon	Negative
Neurologic Disease	Rare	Seizures, dementia, coma	None	Carpal tunnel syndrome	Peripheral Neuropathy
Histological Changes
Mucin on biopsy	No	Yes	Yes	No	Yes
Fibrosis	Dermal, epidermal	Dermal	Dermal	Dermal, hypodermal	Dermal, epidermal
Fibrocytes	No (possible)	Yes	No	No	Yes
Inflammation	Perivascular	Perivascular	No	Yes, with/without eosinophils	No
Clinical Associations		Monoclonal gammopathy	Infection, monoclonal gammopathy, diabetes	Morphea, immune-mediated cytopenias, hematologic and solid malignancies	Acute or chronic renal failure, renal transplant, exposure to gadolinium-based contrast agents