Scleroderma mimics

Scleroderma is named for the presence of its most characteristic quality, namely, skin sclerosis, in addition to symptoms that are commonly encountered in a general population such as Raynaud phenomenon and gastroesophageal reflux. Therefore, having an appropriate level of suspicion for the diagnosis will help facilitate getting patients to the appropriate specialist. However, there are other patients who present with features considered to be typical of scleroderma, such as Raynaud and skin thickening, and who may have a syndrome mimicking scleroderma. The new American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for systemic sclerosis necessitates exclusion of scleroderma mimics (van den Hoogen et al. Ann Rheum Dis. 2013;72(11):1747-55). So familiarity with these mimickers is critical for rheumatologists and other physicians who evaluate patients with scleroderma. Raynaud phenomenon and/or digital ischemia can be associated with multiple different etiologies and rheumatic and non-rheumatic should be considered. Skin thickening (including skin fibrosis) may be seen in a variety of conditions ranging from minor skin irritations (i.e., lichenification from scratching) to a number of systemic diseases that require expert evaluation (scleredema, scleromyxedema). This chapter will focus on the differential diagnosis of these two common presentations, Raynaud phenomenon and skin thickening.