Systemic Sclerosis Mimics

Abstract

Scleroderma-like or pseudo-scleroderma disorders are a wide group of diseases with various etiologies that lead to skin fibrosis therefore mimicking systemic sclerosis. Although they all have skin thickening in common, the distribution, pattern, and character of the involvement as well as systemic manifestations vary between them. The importance of recognizing these mimics resides in their different prognoses and treatment options. A complete clinical history, detailed physical exam as well as laboratory studies and skin biopsy can help differentiate them.

Since the list of scleroderma mimics is long, this chapter will focus on a few key disorders (eosinophilic fasciitis, scleredema, scleromyxedema, nephrogenic systemic fibrosis, diabetic cheiroarthropathy, and lipodermatosclerosis). Other rare conditions, such as metabolic diseases (phenylketonuria, porphyria cutanea tarda), exposure to chemical agents such as bleomycin, vinyl chloride, tryptophan and toxic oil syndrome, and genetic diseases such as progeria, Werner’s syndrome, and Stiff Man Syndrome, which can also cause skin sclerosis will not be discussed.

The absence of typical SSc features such as Raynaud’s phenomenon, antinuclear antibodies, and nailfold capillary changes should raise the index of suspicion for an alternative diagnosis.