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Progressive hemifacial atrophy with localized scleroderma.

E Tan1, N Kürkçüoğlu, M Atalağ

  • 1Department of Neurology, Faculty of Medicine, University of Hacettepe, Ankara, Turkey.

European Neurology
|January 1, 1989
PubMed
Summary
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Localized scleroderma can present with facial hemiatrophy and tongue atrophy. Histological and CT findings confirmed severe muscle atrophy, with EMG studies ruling out neurogenic or myogenic causes.

Area of Science:

  • Connective tissue diseases
  • Dermatology
  • Neurology

Background:

  • Localized scleroderma is a rare autoimmune condition affecting the skin and subcutaneous tissues.
  • Facial hemiatrophy and tongue atrophy are uncommon manifestations of localized scleroderma.

Observation:

  • A patient presented with localized scleroderma accompanied by homolateral tongue atrophy and ipsilateral facial hemiatrophy.
  • Histological examination confirmed the atrophy of tongue and facial muscles.
  • Computed tomography (CT) scans revealed severe atrophy in these facial structures.

Findings:

  • The observed atrophy was severe and histologically confirmed.
  • Electromyography (EMG) studies did not indicate a neurogenic or myogenic origin for the muscle atrophy.

Related Experiment Videos

  • The findings suggest a potential link between localized scleroderma and non-neurogenic, non-myogenic muscle atrophy.

    • Implications:

    • This case highlights the potential for localized scleroderma to cause significant facial and lingual muscle atrophy.
    • Further research is needed to understand the pathogenesis of this association.
    • Clinicians should consider localized scleroderma in the differential diagnosis of facial hemiatrophy and tongue atrophy.