Scleroderma-like fibrosing disorders

Abstract

Many conditions presenting with clinical hard skin and tissue fibrosis can be confused with systemic sclerosis (scleroderma). These disorders have very diverse etiologies and often an unclear pathogenetic mechanism. Distinct clinical characteristics, skin histology, and disease associations may allow one to distinguish these conditions from scleroderma and from each other. A prompt diagnosis is important to spare patients from ineffective treatments and inadequate management. This article highlights nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy), eosinophilic fasciitis (Shulman's syndrome), scleromyxedema, and scleredema. These often are detected in the primary care setting and referred to rheumatologists for further evaluation. Rheumatologists must be able to promptly recognize them to provide valuable prognostic information and appropriate treatment options for affected patients.

Figure 1

Nephrogenic systemic fibrosis. (A) Dense dermal fibrosis is present under a normal epidermis. Disorganized collagen bundles are separated by large clefts and surrounded by numerous fibroblast-like (spindle) cells. Minimal perivascular inflammatory infiltrate is present. (B) The infiltrative process is deep, extending into subcutaneous adipose interlobular septa.

Figure 2

Eosinophilic fasciitis. (A) Patient presenting bilateral involvement of upper extremities with typical “woody” induration of the skin and “puckering”. (B) The same patient after 12 months of corticosteroids treatment.

Figure 3

Scleromyxedema. Patient with classic papular-waxy skin eruption of the face

Figure 4

Scleredema. Patient with diabetes mellitus type 2 and skin induration of the neck and upper back