Early-onset sarcoidosis mimicking refractory cutaneous histiocytosis

Pediatr Blood Cancer. 2008 Mar;50(3):723-6. doi: 10.1002/pbc.21173.

Abstract

A 10-year-old female was diagnosed as having early-onset sarcoidosis (EOS) after a prolonged skin disease. A granuloma emerged on the face at age 2 and massive lesions extended to the rest of the body. Repeated biopsies indicated histiocytic proliferation. At age 7, fever, disseminated macular eruptions, and multinucleated giant cells in the bone marrow prompted vinblastine and prednisolone therapy. Five months after stopping therapy, hypercalcemic crisis occurred along with fever, cytopenias, and interferon-gamma-nemia indicating a macrophage activation syndrome. A biopsy of nodules confirmed the diagnosis of sarcoidosis. The atypical EOS should be differentiated from histiocytosis.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Child
  • Diagnosis, Differential
  • Diagnostic Errors*
  • Early Diagnosis
  • Etoposide / therapeutic use
  • Facial Dermatoses / etiology
  • Female
  • Histiocytosis / diagnosis*
  • Humans
  • Hypercalcemia / etiology
  • Interferon-gamma / blood
  • Interleukins / blood
  • Macrophage Activation
  • Methotrexate / therapeutic use
  • Prednisolone / therapeutic use
  • Sarcoidosis / complications
  • Sarcoidosis / diagnosis*
  • Sarcoidosis / drug therapy
  • Sarcoidosis / pathology
  • Skin Diseases / complications
  • Skin Diseases / diagnosis*
  • Skin Diseases / drug therapy
  • Skin Diseases / pathology
  • Vinblastine / therapeutic use

Substances

  • Interleukins
  • Vinblastine
  • Etoposide
  • Interferon-gamma
  • Prednisolone
  • Methotrexate