engleski

A case of multiple calvarial eosinophilic granulomas

Introduction. Eosinophilic granuloma represents one of a triad of lesions encompassing a disease under the generic name, histiocytosis X or Langerhans cell histiocytosis. The etiology remains uncertain and could be related to undefined immunologic disturbance. It is disease of children and adolescents that very rarely affects adults, representing almost the localized form of a pathological proliferation of histiocytes in bones. The skull is the most common presentation of the disease, and the associated symptoms depend on the location of the lesion. However, multiple lesions of the skull are rare. Clinical details. A 25-year-old woman was referred to our hospital because of headache over the period of three years, that manifested in parietal region often accompanied by swelling of galeal soft-tissue. X-ray of the head revealed multiple ovoid and rounded well-limited lytic calvarial lesions particularly found in the frontal and parietal bones. The diameter of the lesions ranged from 15 to 70 mm. CT scan confirmed these lytic lesions ; a tail of dural enhancement and reactive changes in the overlying galea were not seen. Radionuclide bone scan showed increased uptake at the mentioned sites of the skull. No other localizations of lesions were found. A left frontal craniectomy was performed only for resection of one lesion. The dura mater of that site was also removed and was replaced with a dural patch. Pathologic findings. Accumulations of histiocytes admixed with eosinophils, lymphocytes, plasma cells, and neutrophils infiltrated the bone, galeal skeletal muscle and fibrous tissue, and also the dura mater. Occasionally patchy zones of hemorrhage and necrosis were present. Conclusion. Although the natural history of multiple eosinophilic granulomas remain poorly defined, surgical treatment still remains the mainstay of the therapy. In addition, the different adjuvant treatments is suggested.

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