Etiology • Unknown • Proliferation of Langerhans’ cells (immune surveillance cells)normally found in skin, mucosa, bone marrow, and lymph nodes Clinical Presentation
• A broad spectrum, typically divided into three subsets, as follows: • Unifocal or multifocal chronic disease of bone (eosinophilic
granuloma) • Widely disseminated chronic disease of bone and soft tissue
(Hand-Schüller-Christian disease) • Acute, disseminated disease with bone marrow involvement
(Letterer-Siwe disease) • Most arise in childhood; eosinophilic granuloma often arises in
adolescents and adults. • Jaw lesions noted in up to 20% of cases with tenderness, loose
teeth (focal to segmental), gingival inflammation, and friability Radiographic Findings
• Bone lesions often punched out, sharply circumscribed • “Floating teeth” appearance with alveolar bone involvement • Skeletal survey should be performed to rule out multiple bone
involvement Diagnosis
• Radiographic demonstration of lytic bony lesions • Infiltrate of mononuclear cells, often with clefted nuclei • Often accompanied by a variety of other cell types, including
eosinophils, lymphocytes, giant cells, plasma cells • Immunohistochemical demonstration of CD1a staining • Langerhans’ cells also stain for S-100 protein, although the
antibody is less specific. • Ultrastructural demonstration of cytoplasmic racquet-shaped
Birbeck granules Differential Diagnosis: Clinical
• Cat-scratch disease • Juvenile xanthogranuloma
Differential Diagnosis: Radiologic • Juvenile periodontitis, endocrinopathies, hypophosphatasia,
leukemia, bony malignancy (primary/metastatic) • In adults: myeloma
Treatment • Localized variant
• Surgical curettage of bony lesions • Low-dose radiation therapy of inaccessible lesions
• Widespread variants • Chemotherapy including methotrexate, vincristine,
cyclophosphamide • Bone marrow transplantation for resistant/recurrent disease
Prognosis • Varies with form of disease, as follows:
• Localized variant: very good • Disseminated variant: fair to poor
leukemia, bony malignancy (primary/metastatic) • In adults: myeloma
Treatment • Localized variant
• Surgical curettage of bony lesions • Low-dose radiation therapy of inaccessible lesions
• Widespread variants • Chemotherapy including methotrexate, vincristine,
cyclophosphamide • Bone marrow transplantation for resistant/recurrent disease
Prognosis • Varies with form of disease, as follows:
• Localized variant: very good • Disseminated variant: fair to poor