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