Eosinophilic Granuloma (Subscribe)  

# 60-80% of histiocytosis X
# age 5 - 10 yrs
# presentation: bone pain, local swelling, irritability
# bones
* 50 - 75% solitary / monostotic
* skull/mandible (50%): "punched-out" lucencies, "hole within a hole", "button sequestrum", "floating teeth"
* spine/pelvis (25%): vertebra...

Wheeless' Textbook of Orthopaedics - Discussion: - most common in the thoracic spine; - may present with progressive back pain; - vertebra plana: - in 2-6 yrs old child, EOG may present w/...

Objectives Discuss the relationship and/ or nomenclature of eosinophilic granuloma, Letterer-Siwe disease, Hand-Schuller-Christian disease, histiocytosis X, and Langerhans-cell granulomatosis Discuss the bones most frequently affected by eosinophilic granuloma Discuss treatment of spine lesions (vertebra plana) associated with eosinophilic granuloma

Illustrated cases

Discussion: - includes a group of disorders characterized by a variety of tumor like lesions, which arise from from clonal proliferation of Langerhans-type histiocytes; - most common in children less than 12...

Also known as solitary Langerhan's cell histiocytosis (LCH), eosinophilic granuloma is the most benign member of a group of disorders of the reticuloendothelial system, including Hand-Schuller-Christian disease and disseminated LCH. Langerhans cell histiocytosis is a spectrum of diseases that primarily affects the skeleton but can involve the reticuloendothelial system...

chronic diseminated form of histiocytosis X
* age 1 - 3 yrs most common
* skull and mandible lesions similar to EG, but more numerous
* "geographic skull", "floating teeth"
* adenopathy
* enlarged liver and spleen
* skin lesions
* diabetes insipidus
* exophthalmos
* lung disease

6 synonyms or equivalents were found. Histiocytosis X aka/or Langerhans cell histiocytosis aka/or Letterer-Siwe syndrome aka/or Schueller-Christian disease aka/or Eosinophilic granuloma aka/or Hand-Schuller-Christian disease may cause or feature Miscellaneous syndromes Bone cyst Bone lysis Hepatocellular jaundice Orbital mass Symptoms and Signs Breathlessness Exophthalmos Gum pathology Haemoptysis Hepatomegaly Lymphadenopathy Mastoiditis Oral ulceration Platyspondyly Rash Short stature Splenomegaly Suppurative otitis media Tooth loss Haematological abnormalities Leucoerythroblastic anaemia Neutropenia Red cell production reduced Thrombocytopenia Biochemical abnormalities Diabetes insipidus, non-nephrogenic Hepatocellular jaundice Histopathological abnormalities Pulmonary fibrosis X-ray abnormalities Periosteal reaction Endocrine conditions Hypopituitarism Malignant neoplastic conditions Bone metastases Respiratory conditions Pneumothorax Pulmonary fibrosis Histiocytosis X:...

Histiocytosis X (Lichtenstein 1977) or Langerhan's Cell histiocytosis (Bergholz 1979), is the currently favored term, for a spectrum of granulomatous conditions characterized by proliferating histiocytic cells admixed with eosinophils and other inflammatory cells.
The purpose of this chapter is to familiarize the reader with the spectrum of conditions encompassed under...

The Langerhans cell is a dendritic cell of the epidermis that was first described by a medical student, Paul Langerhans, who thought it was part of the nervous system (Langerhans, 1868). Birbeck et al. (1961) found that the Langerhans cell displays a unique electron-microscopic morphology. The discoveries that these cells...

Account of the familial incidence of LS Disease and references

Acute diseminated form of histiocytosis X
* age 0 - 1 yr
* worst prognosis (70% mortality)
* visceral involvement: hepatosplenomegaly
* purpura, anemia, lymphadenopathy
* bone: extensive lytic skull lesions, "raindrop" skull

# females, middle age
# deposition arthropathy
# bilaterally symmetric involvement of hands
# soft-tissue swelling
# well-circumscribed marginal bony erosions

eosinophilic granuloma