Langerhans cell histiocytosis

Langerhans cell histiocytosis(LCH)는 hystiocytes의 침윤으로 인한 single or multiple osteolytic bone lesions을 특징으로 하는 질환이다.

임상 양상

• histiocytes(lymphocytes, macrophages, and eosinophils)은 거의 모든 기관에 침윤이 가능(흔히 심장과 심장을 제외한 skin, lymph nodes, lungs, thymus, liver, spleen, bone marrow, or central nervous system 등)
• bones(80%): punched out lesion, beveled edge, vertebra plana
• skin(30-60%):infant, maculopapular rash
• thymus: enlargement.
• lung: multiple cysts

참고

• hemophagocytic lymphohistiocytosis

DDx

• other osteolytic skull lesion
  • dermoic cyst, eosinophilic granuloma, metastasis, surgical defects