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Case 11

History:

22 year old male with neck pain

Findings:

Lateral radiograph showing vertebra plana manifested by marked flattening of the C6 vertebral body with relatively preserved intervertebral disk space and lack of kyphosis.

Diagnosis:

Eosinophilic granuloma (EG)

Discussion:

Eosinophilic granuloma (EG) is characterized by single or multiple skeletal lesions occurring predominantly in children, adolescents and young adults. It represents 70 percent of the total number of causes of Histiocytosis X and is more common in males than females. Clinical manifestations include local pain, tenderness sometimes a palpable soft tissue mass. Fever and leukocytosis may also be present. Common sites include skull, mandible, ribs, spine and long bones. Vertebral destruction can lead to a flattened vertebral body, termed “Vertebra Plana”, more frequent in children. EG can produce bubbly, lytic, expansile lesions of both the body and posterior elements. The intervertebral disc is usually normal. A single lytic defect must be differentiated from neoplastic and inflammatory lesions as well as fibrous dysplasia. Multiple lytic lesions may simulate infection, metastasis, lymphoma, leukemia, hyper-parathyroidism with brown tumors, and Gaucher’s disease. With proper treatment, in young patients if lesions spontaneously regress, vertebral height is often partially restored. The degree of recovery is typically greater when the patient is young.

Differential Diagnosis:

multiple myeloma, metastatic disease, Ewing sarcoma, lymphoma, leukemia, Gaucher disease, TB

References:

  • Resnick. Orthopedics: Diagnosis of Bone and Joint Disorders. 3rd edition. 1994; volume 4; p2429-2439.
  • Bhatia S, Nesbit M Jr, Egeler RM, et al. Epidemiological study of Langerhans cell histiocytosis in children. J Pediatr 1997; 130(5): 774-784
  • Huang F, Arceci R. The histiocytoses of infancy. Semin Perinatol 1999; 23(4): 319--331.
  • Agrawal V; Nityanand S. Langerhans Cell Histiocytosis. Journal of Association of Physicians of India. Mar 2000 48; (3); p 346 - 8 ISSN: 0004- 5772
  • Floman Y; Bar-On E; Mosheiff R; Mirovsky Y; Robin GC; Ramu N. Eosinophilic Granuloma of Spine. Journal of Pediatric Orthopedics. Part B (United States). Oct 1997 6; (4); p260 - 5 ISSN: 1060 - 152X.
  • C Villas et al. Eosinophilic Granuloma of the Spine with and without Vertebral Plana ; long term follow up of six cases, Journal of Spinal Disorders, 1993 6; (3); p260 - 8.