[Carpal Extensor Tenosynovitis and Adult Sickle Cell Vaso-Occlusive Crisis]

N Vaserman1, N Boizan1, S Remadjidobian2

  • 1Service de médecine interne-rhumatologie, CHC Cayenne, BP 6006, 97306 Cayenne Cedex, Guyane française, France.

Insights

Hand-foot syndrome, typically seen in young children, occurred in a 36-year-old adult with sickle cell disease (SS). This rare presentation during a vaso-occlusive crisis highlights a less recognized complication of sickle cell anemia.

Area of Science:

  • Rheumatology
  • Hematology
  • Pediatrics

Background:

  • Osteoarticular complications are common in sickle cell anemia.
  • Hand-foot syndrome (dactylitis) is a characteristic manifestation typically observed in children under four years old.
  • Its occurrence in adults is exceptionally rare.

Purpose of the Study:

  • To report a unique case of hand-foot syndrome in an adult with sickle cell disease (SS).
  • To describe the clinical presentation and diagnostic considerations for this rare manifestation.
  • To emphasize the importance of considering vaso-occlusive complications in adult sickle cell disease.

Main Methods:

  • Case report of a 36-year-old adult with homozygous sickle cell disease (SS).
  • Clinical observation of hand-foot syndrome during a vaso-occlusive crisis, presenting as tenosynovitis of carpal extensors.
  • Differential diagnosis to exclude infectious etiologies of palmar tenosynovitis.

Main Results:

  • The adult patient presented with tenosynovitis mimicking infection but was diagnosed with hand-foot syndrome.
  • The vaso-occlusive origin of the tenosynovitis was confirmed by the rapid clinical improvement.
  • This case expands the known age range for hand-foot syndrome in sickle cell disease.

Conclusions:

  • Hand-foot syndrome can occur in adult patients with sickle cell disease (SS), albeit rarely.
  • Vaso-occlusive crises should be considered in the differential diagnosis of tenosynovitis in adults with sickle cell disease.
  • This case underscores the diverse clinical spectrum of sickle cell anemia complications.

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