Orthopaedic MD

eosinophilic granuloma

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/ collapse of single vertebra; - diff dx of single level collapse: - ewing's sarcoma; - ABC - osteomyelitis (look for loss of disc height); - ref: Vertebra plana of the lumbar spine caused by an ABC: a case report. PJ Papagelopoulos MD et al. The American Journal of Orthopedics. Feb 1999. p 119.

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

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 years of age (but can occur in young adults); - etiology: - occurs as a result of metabolic defects in the reticuloendothelia system; - it is sometimes non painful, unless of course a fracture occurs; - hallmark is presence of an osseous lesion (70-90%) most frequently arising in skull and femur; - osseous lesions may produce rapidly destructive bone lesions; - soft tissue masses associated w/ skeletal lesions rarely are seen, - disease sub-types: - note that intermediate forms of the disease occur and individual disease presentations may change from one form to another; - Histiocytosis X; - Hand Schuller Christian disease - occurs in children> 3 yrs; - traid of skull lesions, exophthalmos, & diabetes insipidus; - a minority of patients will have wide spread viceral involvement; - cranial lesions are always present in this disease; - Letterer Siwe dz; - represents more acute manifestations of histiocytosis & generally has an age of onset of less than 3 yrs; - look for recurrent bacteremia, diffuse lymphadenopathy, & skin lesions; - disease is commonly fatal; - poor prognostic signs: - involvement at young age, rapid disease progression, organ involvement (eg pituitary, lung, hematopoietic, or liver involvement); - organ dysfunction carries an especially poor prognosis;

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: Definition(s) via UMLS: - "The most benign clinical form of Langerhans-cell histiocytosis (HISTIOCYTOSIS, LANGERHANS-CELL), which involves localized nodular lesions of the gastric mucosa, small intestine, bones, lungs, or skin, with infiltration by eosinophils. The proliferating cell that appears to be responsible for the clinical manifestations is the Langerhans cell."

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 broad designation of Histiocytosis X, with a recommended plan of management for each condition. Emphasis will be given to discussion and management of the condition most commonly referred to as Eosinophilic Granuloma. John L. Eady, MD, FACS Courtney Gleason University of South Carolina School of Medicine Columbia, SC Outline - I. Introduction II. Historical Perspective III. Anatomic and Physciologic Considerations IV. Natural History and Classifications V. Diagnosis and Recognition Clinical Presentation Radiographic Findings Gross and Microscopic Appearance Differential Diagnosis VI. Treatment Methods of Management Spine Pelvis Sacral Long Tubular Weight Bearing Bones (femur, tibia, humerus) VII. Summary X. References XI. Patient Education Summary

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 are not confined to skin and that they make up a sizable portion of the cellular infiltrate in histiocytosis X (246400), along with other evidence, suggested that they play an immunologic role in protecting against environmental antigens. Egeler and D'Angio (1995) presented a classification of histiocytosis syndromes in children: class I, Langerhans cell histiocytosis (LCH); class II, histiocytosis of mononuclear macrophages other than Langerhans cells, including familial hemophagocytic lymphohistiocytosis (267700); and class III, malignant histiocytic disorders, including histiocytic lymphoma.

Account of the familial incidence of LS Disease and references

eosinophilic granuloma