Langerhans cell histiocytosis

Case contributed by Dr Prashant Mudgal

Presentation

Young child presented with multiple soft tissue swelling on scalp, pyrexia of unknown origin and gait difficulties and painful limp.

Patient Data

Age: 5 years
Gender: Female
X-ray

Multiple well defined  punched out osteolytic lesions with scalloped edges (geographic skull) are seen in bilateral parital regions. The lesions have a characteristic bevelled edge.

CT

There are multiple osteolytic lesions causing full thickness bone destruction seen in bilateral parietal bones.

MRI

There are abnormal well defined heterogenously enhancing lobulated soft tissue lesions of altered signal intensity along the right iliac blade, bilateral ischium and left femoral diaphysis.. The lesion is causing cortical erosion of the adjacent iliac bone .The lesion appears hypointense on T1W , heterogenously hyperintense on T2 and STIR.

The visualised muscles, bones and rest of the pelvic organs are normal.

The imaging features of the skull, pelvic bones and femoral lesions are most likely suggestive of Langerhans cell histiocytosis

Case Discussion

Histiocytosis X is most often seen in children ages 1 to 15  with the peak incidence at 1-4
years of age.

The rate peaks among children ages 5 to10 years. It most commonly affects skull among the flat bones and femur among the long bones. The diagnostic tests that can be carried out in children are: 

  • Skin biopsy for the presence of Langerhans cells
  • Bone marrow biopsy for the presence of Langerhans cells
  • Complete blood count
  • X-rays skeletal survey to find out number of affected bones.
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Case information

rID: 28345
Case created: 22nd Mar 2014
Last edited: 14th Mar 2016
Tag: pm
Inclusion in quiz mode: Included

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