September 20, 2018
A 16-year-old girl with progressive headache, cutaneous reddening, and itching in the left cranial vault; blood exams show increased inflammatory markers.
Langerhans cell histiocytosis (Eosinophilic granuloma)
- Background
- The skeleton is the most common location for single-lesion Langerhans cell histiocytosis (LCH), often referred to as eosinophilic granuloma;
- It primarily occurs in older children and young adults;
- Any bone can be involved. The more common sites include the skull, mandible, spine, ribs, and the long bones.
- Clinical Presentation
- Solitary bone lesion may be asymptomatic;
- Patients may present with local pain, swelling and tenderness;
- Fever and leucocytosis may occur.
- Key Diagnostic Features
- The radiologic appearance depends on the site of involvement and the phase of the disease;
- In the skull, solitary or multiple punched out lytic lesions without sclerotic rim. The uneven destruction of the outer and inner cranial tables results in a beveled-edge or double-contour appearance. An enhancing soft tissue component may be identifiable;
- Pathology demonstrates proliferation of Langherans cells with an abundance of eosinophils, lymphocytes and neutrophils.
- Differential Diagnosis
- Osteomyelitis
- Primary bone tumors
- Metastases
- Lymphoma or leukemia
- Treatment
- Local treatment (excision and curettage);
- Intralesional instillation of steroids may be effective for a limited number of bony lesions;
- Low dose radiation or systemic therapies (indomethacin, bisphosphonates, or chemotherapy) should be restricted to emergency situations (such as optic nerve compression) and more extensive disease.
