Medical Conditions and Exposures Associated with Cutaneous Sclerosis

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Medical Conditions and Exposures Associated with Cutaneous Sclerosis

Disease mimics

  • • Localized scleroderma syndromes
    • • Morphea
    • • Linear scleroderma
  • • Scleredema
  • • Scleromyxedema
  • • EF
  • • NSF
  • • Chronic graft-versus-host disease (cGVHD)
  • • POEMS syndrome—polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes
  • • Pachydermoperiostitis
  • • Metabolic diseases
    • • Diabetic cheiroarthropathy (see Chapter 48 : Endocrine-Associated Arthropathies)
    • • Hypothyroidism (myxedema)
    • • Porphyria cutanea tarda
    • • Phenylketonuria
  • • Paraneoplastic disorders
  • • Systemic amyloidosis
  • • Complex regional pain syndrome (late stage; see Chapter 65 : Complex Regional Pain Syndrome)
  • • Lipodermatosclerosis
  • • Genetic disorders (e.g., Werner syndrome)

Medication/treatment-related

  • • L-tryptophan (eosinophilia-myalgia syndrome [EMS])
  • • Aniline-denatured rapeseed oil (toxic oil syndrome)
  • • Bleomycin
  • • Pentazocine
  • • Carbidopa
  • • Melphalan
  • • Docetaxel (taxotere)
  • • Post-radiation fibrosis

Occupational exposure

  • • Vinyl chloride
  • • Silica
  • • Pesticides (malathion, diniconozole)
  • • Organic solvents (benzene, toluene, and others)
  • • Epoxy resins

Recognition of the various causes of cutaneous sclerosis is especially important in the evaluation of a patient who lacks characteristic features of SSc. In such patients, consideration should be given to skin biopsy as well as ruling out potential exposures, underlying neoplasm or plasma cell dyscrasia, as well as the other medical conditions listed above.

Several of the more common conditions rheumatologists encounter that mimic SSc (morphea, scleredema, scleromyxedema, eosinophilic fasciitis, NSF, and cGVHD) will be discussed.

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