Several features can help differentiate SSS and scleredema. In SSS, disease is centered on or around the pelvic and shoulder girdle, whereas scleredema is centered on the face, head, neck, and back24 and with rare exception25 only involves the pelvic or shoulder girdle with concomitant involvement of the face, neck, or upper back.24 Joint restriction, although common in SSS, is rare in scleredema and is more often due to a “bulk” effect of the thick skin usually affecting the eyelids, neck, mouth, and shoulder.18,26- 29 The skin stiffness in SSS, in our experience, is often well demarcated, uneven, or lumpy and may not involve an entire anatomic unit, while the stiffness in scleredema is more uniform, typically encompassing an entire involved anatomic unit. The abrupt onset often seen in scleredema is not a feature of SSS. The histopathologic features overlap, but increased fibroblast cellularity, thickened collagen bundles in a horizontal arrangement, and diminished spaces between individual bundles, if present, can help differentiate biopsy results of SSS from scleredema. Typically in scleredema, the collagen bundle pattern is nearly normal.