Histocytoses-X :-

It is disease that primarily affects bone but occasionally may also affect other organ system and present in multisystemic pattern.

It is a group of idiopathic disorders characterized by clonal proliferation of specialized bone marrow.

It is also known as Langerhans Cell Histiocytosis (LCH). It is dendritic cell disorder

Clinical features :-

  • Pain, swelling or lump in a bone that does not go away.
  • Broken bone from only a minor injury or for no apparent reason.
  • Loose teeth (when not expected) or swollen gums.
  • Ear infection, cysts in the ear or fluid oozing from the ear.
  • Skin rash, such as on the scalp or buttocks.

Clinical syndromes :-

  • Letterer-siwe disease
  • Eosinophilic granuloma
  • Hand-schuller-christian disease

Histopathology :-

  • Convoluted vesicular chromatin is seen
  • Small nucleolus is seen
  • Light blue dendritic is seen
  • Convoluted peripheral heterochromatin is seen

Treatment :-

  • Steroid therapy, such as prednisone, is used to treat LCH lesions.
  • Bisphosphonate therapy (such as pamidronate, zoledronate, or alendronate) is used to treat LCH lesions of the bone.
  • Local curettage or excision of lesions. Heal on it’s own. Prognosis is good.

Reference :-

Image :- Google
Writing :- notes made from mastering bds and Sanjay Kumar Purkait books

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