Localized Fibrosing Disorders - Linear Scleroderma, Morphea, and Regional Fibrosis Differential Diagnoses

Updated: Feb 23, 2018
  • Author: Mariana J Kaplan, MD; Chief Editor: Herbert S Diamond, MD  more...
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DDx

Diagnostic Considerations

Distinguishing eosinophilic fasciitis (ET) from systemic sclerosis (SSc) is important because corticosteroids are first-line therapy for EF, but are generally avoided in patients with SSc due to associations with renal crisis. Furthermore, early treatment of EF has been reported to improved outcomes. Both disorders frequently present with induration of the extremities but visceral involvement in EF is generally limited to hematologic abnormalities, and thus an extensive systemic workup is not indicated as it is in SSc. Clinically, nailfold capillary changes and Raynaud phenomenon are typically absent in EF, unlike in SSc, and skin tightening on the distal digits is lacking. [5]  

Other problems to consider in the differential diagnosis include the following:

  • Acrodermatitis chronica atrophicans

  • Bleomycin exposure

  • Bromocriptine exposure

  • Carcinoid syndrome

  • Panniculitis

  • Ergot exposure

  • Localized lipoatrophies

  • Methysergide exposure

  • Phenylketonuria

  • POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes)

  • Poikiloderma

  • Porphyria cutanea tarda

  • Scleredema

  • Scleromyxedema

  • Vitamin K injection

Differential Diagnoses