The Differential Diagnosis of Systemic Sclerosis

Alan Tyndall; Susanna Fistarol

Disclosures

Curr Opin Rheumatol. 2013;25(6):692-699. 

In This Article

Disabling Pansclerotic Morphea

A rare and sometimes fatally progressive disorder mainly in children, occasionally in early middle-aged persons which is associated with chronic recalcitrant ulcers of the skin (Fig. 9) and increased risk of malignant transformation with the development of nonmelanoma skin cancer, disabling pansclerotic morphea involves all layers of the skin, subcutaneous tissue, fascia, muscle, tendon and bone, leading to joint contractures, musculoskeletal atrophy and immobility. Despite often extreme skin thickening involving the entire skin including the face, but sparing the fingers, no systemic or internal organ involvement occurs. There are no vascular abnormalities or autoantibodies, and skin biopsy shows only fibrosis without eosinophils or increased mucin. There is no proven effective treatment.

Figure 9.

A 40-year-old man with pansclerotic morphea. Note thickened skin on his forearm, but no finger involvement or Raynaud's phenomenon. Frenulum thickening typical for SSc is not present (arrow). SSc, systemic sclerosis.

Other diffuse skin thickening syndromes include sclerodermiform premature aging syndromes, such as progeria, acrogeria or Werner's syndrome, chronic venous stasis (lipodermosclerosis), thyroid disease (hypoactivity and hyperactivity), acrodermatitis chronic atrophicans, chronic graft versus host disease, primary amyloidosis, sclerodermoid porphyria cutanea tarda and toxic reactions such as eosinophilia–myalgia syndrome (L-tryptophan) and Spanish toxic oil syndrome (denatured rapeseed oil). These are mostly easily distinguished by the history and accompanying features.

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