[Febrile pseudotumor lesion of the arm. Atypical presentation of Shulman syndrome]]

Presse Med. 2003 Mar 22;32(11):498-500.
[Article in French]

Abstract

Introduction: The diagnostic approach when confronted with a pseudo-tumoral lesion and fever is difficult since it evokes an infectious, rheumatismal inflammatory or even systemic disease.

Observation: A 39 year-old Vietnamese man was hospitalised for polyarthralgia if the arm and fever (39 degrees C). He was treated with non-steroidal anti-inflammatories, which were only partially effective. Biological examinations revealed a severe inflammatory syndrome and cytolysis three-fold higher than normal. Since the infectious, rheumatismal and immunological explorations were negative and in view of the appearance of a pseudo-tumoral inflammatory lesion of the left arm concomitant to infiltration of the underlying muscle revealed on MRI, a muscle biopsy was performed showing eosinophilic fasciitis. Diagnosis of Shulman's syndrome was made and the affection rapidly improved with corticosteroids.

Comments: The presentation of our patient was atypical for several reasons: his Vietnamese origin, the initial fever peak at 39 degrees C, the unilateral involvement, the severe inflammatory syndrome, the initial absence of hypereosinophilia, the visceral involvement and notably myositis are all uncommon in Shulman's syndrome. Hence, it is important to rule out its differential diagnosis from local edematous scleroderma, which does not share the same prognosis.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Adult
  • Arm* / pathology
  • Arthralgia / etiology*
  • Biopsy
  • Diagnosis, Differential
  • Eosinophilia / diagnosis*
  • Eosinophilia / pathology
  • Fascia / pathology
  • Fasciitis / diagnosis*
  • Fasciitis / pathology
  • Fever of Unknown Origin / etiology*
  • Granuloma, Plasma Cell / diagnosis*
  • Granuloma, Plasma Cell / pathology
  • Humans
  • Magnetic Resonance Angiography
  • Male
  • Muscle, Skeletal / pathology
  • Syndrome