Hypothenar hammer syndrome followed by systemic sclerosis

J Rheumatol. 2000 Oct;27(10):2516-7.

Abstract

We describe the first case of bilateral hypothenar hammer syndrome (HHS) followed by systemic sclerosis (SSc) that was associated with silica exposure (Erasmus syndrome). The patient was a woman smoothing tiles in an earthenware factory who presented with bilateral digital ischemia associated with Raynaud's phenomenon. HHS was diagnosed, based on an angiographic study showing aneurysm of the ulnar arteries and occlusions of multiple digital arteries. Pulmonary silicosis was also diagnosed on pulmonary tomodensitometry. Two years later digital swelling with acroosteolysis developed. The FANA test was positive (titer 1:640) and anticentromere antibody tests were also positive. Esophageal manometry showed dysmotility of the lower esophagus. These findings were consistent with a diagnosis of SSc.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aneurysm / diagnostic imaging
  • Aneurysm / etiology*
  • Angiography
  • Cumulative Trauma Disorders / diagnostic imaging
  • Cumulative Trauma Disorders / etiology*
  • Female
  • Hand / blood supply*
  • Hand / pathology
  • Humans
  • Radiography, Thoracic
  • Raynaud Disease / etiology
  • Scleroderma, Systemic / diagnostic imaging
  • Scleroderma, Systemic / etiology*
  • Silicosis / diagnostic imaging
  • Silicosis / etiology*
  • Syndrome
  • Ulnar Artery / diagnostic imaging
  • Ulnar Artery / injuries*
  • Ulnar Artery / pathology