Table 1 Distinguishing clinical features of scleroderma-like disorders.
From: The importance of recognizing scleroderma-type disorders in clinical practice
Disorder | Distribution of skin involvement | Quality of skin | Associated clinical features | Associated laboratory features |
|---|---|---|---|---|
Scleroderma | Hands and face common; diffuse subset involves proximal extremities and trunk; mid back spared | Thick, smooth, shiny induration | Raynaud phenomenon; gastroesophageal reflux; dysphagia; dyspnea | Antinuclear antibody positivity; scleroderma-specific autoantibodies |
Scleromyxedema | Scleroderma distribution with prominent findings around glabella, ears and posterior neck | Cobblestone induration with 2–3 mm waxy papules | Dysphagia; Raynaud phenomenon; musculoskeletal pain | Monoclonal gammopathy |
Nephrogenic fibrosing dermopathy | Extremities and trunk; face spared | Deep indurated plaques | Marked flexion contractures; renal failure and/or insufficiency; exposure to gadolinium | N/A |
Eosinophilic fasciitis | Extremities and trunk; hands and feet spared | Woody induration deeper than superficial dermis | Can overlap with plaque morphea | Possible peripheral eosinophilia; immune-mediated cytopenias |
Scleredema | Neck, back and proximal arms | Doughy induration | Discomfort in areas of involvement | Poorly controlled diabetes; recent streptococcal infection; monoclonal gammopathy |
Pansclerotic morphea | Extremities, face, feet; hands spared | Thick induration similar to that of diffuse scleroderma | Contractures; no systemic features of scleroderma | N/A |
