METABOLICDISTURBANCE     S     Prepared by:          Dr. Rea Corpuz
Metabolic Disturbances (1) Histiocytosis X    Hand Schuller Christian    Eosinophilic Granuloma    Letterer Siwe (2) ...
Histiocytosis X Langerhans cell diseases  (LCD)    also formerly known as      • histiocytosis X      • idiopathic histi...
Histiocytosis X Langerhans cell diseases  (LCD)    disorder characterized by      • proliferation of cells exhibiting   ...
Histiocytosis X historically, term histiocytosis  X was used to encompass 3  disorders:    Eosinophilc Granuloma    Sch...
Histiocytosis X grouped together because  of similar microscopic  appearance
Histiocytosis X(Eosinophic Granuloma) also known as Chronic Localized  (LCD) refers to solitary or multiple  bone lesion...
Histiocytosis X(Eosinophic Granuloma) occasionally, gross periodontal  destruction exposes the roots  of teeth adults ar...
Histiocytosis X(Eosinophic Granuloma) Radiographic Features   rounded area of radiolucency    with indistinct margins  ...
Histiocytosis X(Hand-Schuller Christian Syndrome) also known as Chronic Disseminated  (LCD) specific clinical triad of l...
Histiocytosis X(Hand-Schuller Christian Syndrome) many affected persons also  exhibit:    lymphadenopathy    dermatitis...
Histiocytosis X(Letterer-Siwe Disease) also known as Acute  Disseminated LCD malignant process characterized by rapidly...
Histiocytosis X(Letterer-Siwe Disease) aggressive form of histiocytosis affects infants or young children
Histiocytosis X(Letterer-Siwe Disease) widespread organ, bone,  + skin involvement by  proliferative process in infants  ...
Histiocytosis X Etiology & Pathogenesis    unknown    although viral infection     has long been suspected
Histiocytosis X Clinical Features    condition of children +     young adult    age range also extends     to older adu...
Histiocytosis X Clinical Features    monostotic + polyostotic     forms of disorder may     affect any bone of body     ...
Histiocytosis X Clinical Features
Histiocytosis X Clinical Features    oral changes may be     initial presentation in all     forms of disorder    skin,...
Histiocytosis X Clinical Features    tenderness                   common    pain            patient                  co...
Histiocytosis X Clinical Features    loosening of teeth in     area of affected alveolar     bone is common occurrence  ...
Histiocytosis X Clinical Features    oral mucosal lesions in     the form of      • submucosal nodules      • ulcers    ...
Histiocytosis X Clinical Features    jaw      • solitary or multiple       radiolucent lesions      • can affect alveola...
Histiocytosis X Clinical Features    jaw      • bone lesions with a       sharply circumscribed       or punched out app...
Histiocytosis X Treatment & Prognosis   Localized Disease     • Curettage     • Radiation, low dose     • Intralesional ...
Histiocytosis X Treatment & Prognosis   Disseminated Disease     • Immunosuppressive agents     • corticosteroids
Niemann-Pick Disease considered to be as a storage  disease affected patients lack enzymes  necessary for processing  sp...
Niemann-Pick Disease because of this accumulation  it appeared that cells were  attempting to store this  substances; th...
Niemann-Pick Disease characterized by deficiency of  acid sphingomyelinase    resulting in accumulation     of sphingomy...
Niemann-Pick Disease occurs as 3 different types,  each associated with a different  clinical expression + prognosis    ...
Niemann-Pick Disease Type A & B   caused by deficiency of acid    sphingomyelinase Type C   primarily mutation of    N...
Niemann-Pick Disease Type A & C   neuronopathic features     • psychomotor retardation     • dementia     • spasticity  ...
Niemann-Pick Disease Type B   normally survive into    adulthood   exhibit visceral signs   primarily hepatosplenomega...
Niemann-Pick Disease Treatment   poor prognosis   genetic counseling should    be provided for affected    families
References: Books   Cawson, R.A: Cawson’s Essentials of Oral       Oral Pathology and Oral Medicine,       8th Edition  ...
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Metabolic disturbances (1)

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Metabolic disturbances (1)

  1. 1. METABOLICDISTURBANCE S Prepared by: Dr. Rea Corpuz
  2. 2. Metabolic Disturbances (1) Histiocytosis X  Hand Schuller Christian  Eosinophilic Granuloma  Letterer Siwe (2) Niemann Pick Disease
  3. 3. Histiocytosis X Langerhans cell diseases (LCD)  also formerly known as • histiocytosis X • idiopathic histiocytosis
  4. 4. Histiocytosis X Langerhans cell diseases (LCD)  disorder characterized by • proliferation of cells exhibiting phenotypic characteristics of Langerhans cells
  5. 5. Histiocytosis X historically, term histiocytosis X was used to encompass 3 disorders:  Eosinophilc Granuloma  Schuller-Christian Syndrome  Letterer-Siwe Disease
  6. 6. Histiocytosis X grouped together because of similar microscopic appearance
  7. 7. Histiocytosis X(Eosinophic Granuloma) also known as Chronic Localized (LCD) refers to solitary or multiple bone lesions only
  8. 8. Histiocytosis X(Eosinophic Granuloma) occasionally, gross periodontal destruction exposes the roots of teeth adults are mainly affected lesion is most frequently in mandible
  9. 9. Histiocytosis X(Eosinophic Granuloma) Radiographic Features  rounded area of radiolucency with indistinct margins  appearance of floating in air
  10. 10. Histiocytosis X(Hand-Schuller Christian Syndrome) also known as Chronic Disseminated (LCD) specific clinical triad of lytic bone lesions exophthalmos diabetes insipidus
  11. 11. Histiocytosis X(Hand-Schuller Christian Syndrome) many affected persons also exhibit:  lymphadenopathy  dermatitis  splenomegaly  hepatomegaly
  12. 12. Histiocytosis X(Letterer-Siwe Disease) also known as Acute Disseminated LCD malignant process characterized by rapidly progressive, often fatal course
  13. 13. Histiocytosis X(Letterer-Siwe Disease) aggressive form of histiocytosis affects infants or young children
  14. 14. Histiocytosis X(Letterer-Siwe Disease) widespread organ, bone, + skin involvement by proliferative process in infants has been the common presentation
  15. 15. Histiocytosis X Etiology & Pathogenesis  unknown  although viral infection has long been suspected
  16. 16. Histiocytosis X Clinical Features  condition of children + young adult  age range also extends to older adults
  17. 17. Histiocytosis X Clinical Features  monostotic + polyostotic forms of disorder may affect any bone of body • skull • mandible • ribs • vertebrae • longs bone are often involved
  18. 18. Histiocytosis X Clinical Features
  19. 19. Histiocytosis X Clinical Features  oral changes may be initial presentation in all forms of disorder  skin, mucosal, or bone involvement in head + neck region was noted in more than 80% of children
  20. 20. Histiocytosis X Clinical Features  tenderness common  pain patient complaints  swelling
  21. 21. Histiocytosis X Clinical Features  loosening of teeth in area of affected alveolar bone is common occurrence  gingival tissues • inflammed • hyperplastic • ulcerated
  22. 22. Histiocytosis X Clinical Features  oral mucosal lesions in the form of • submucosal nodules • ulcers • leukoplakia
  23. 23. Histiocytosis X Clinical Features  jaw • solitary or multiple radiolucent lesions • can affect alveolar bone causing teeth to appear as if they were floating
  24. 24. Histiocytosis X Clinical Features  jaw • bone lesions with a sharply circumscribed or punched out appearance may also occur in central aspect of  mandible  maxilla
  25. 25. Histiocytosis X Treatment & Prognosis  Localized Disease • Curettage • Radiation, low dose • Intralesional corticosteroid injection • rare spontaneous regression
  26. 26. Histiocytosis X Treatment & Prognosis  Disseminated Disease • Immunosuppressive agents • corticosteroids
  27. 27. Niemann-Pick Disease considered to be as a storage disease affected patients lack enzymes necessary for processing specific lipids results in accumulation of the lipids within a variety of cells
  28. 28. Niemann-Pick Disease because of this accumulation it appeared that cells were attempting to store this substances; therefore the term storage disease was commonly used for these disorders
  29. 29. Niemann-Pick Disease characterized by deficiency of acid sphingomyelinase  resulting in accumulation of sphingomyelin  also within lysosomes of macrophages
  30. 30. Niemann-Pick Disease occurs as 3 different types, each associated with a different clinical expression + prognosis  Type A  Type B  Type C
  31. 31. Niemann-Pick Disease Type A & B  caused by deficiency of acid sphingomyelinase Type C  primarily mutation of NPC-1 • gene involved with cholesterol processing
  32. 32. Niemann-Pick Disease Type A & C  neuronopathic features • psychomotor retardation • dementia • spasticity • hepatosplenomegaly • with death occuring during 1st or 2nd decade of life
  33. 33. Niemann-Pick Disease Type B  normally survive into adulthood  exhibit visceral signs  primarily hepatosplenomegaly  sometime pulmonary involvement
  34. 34. Niemann-Pick Disease Treatment  poor prognosis  genetic counseling should be provided for affected families
  35. 35. References: Books  Cawson, R.A: Cawson’s Essentials of Oral Oral Pathology and Oral Medicine, 8th Edition • (page 165-167 )  Neville, et. al: Oral and Maxillofacial Pathology 3rd Edition • (pages 590-592; 819-820) Regezi, et. al: Oral Pathology: Clinical Pathologic Correlations, 5th Edition • (pages 296-299)

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