Langerhans Cell Histiocytosis
Langerhans
Cell Histiocytosis (LCH) has collected many pseudonyms over the years including
histiocytosis X and eosinophilic granuloma (EG). It has twice the
prevalence in male patients compared to females and strikes in the 5-15
year old range. Lesions are usually solitary but 10% will have two
to three foci. It most commonly affects
the skull but also carries a predilection for the spine. Symptoms are usually
constitutional, such as fever, and patients often have symptoms of pain over
the lesion. Labs are non-specific and
often only present with an elevated ESR.
Radiographic studies show a lytic lesion that can affect any
segment of bone beginning in the diaphysis and progressing to the epiphysis
rarely. Over time, weakening of the
vertebra in the spine will cause flattening of the vertebrae known as “vertebrae planae.” (Figure #12A, 12B,
13) The radiographic appearance can easily be mistaken for Ewing’s sarcoma,
metastatic neuroblastoma, and osteomyelitis. Histologically these lesions show large histiocytes speckled with
eosinophils (Figure #11). The
eosinophils were once thought to be a primary determinate of the disease (thus
the name eosinophilic granuloma), but are now thought to be secondary
findings.
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Figure
# 11
Langerhan’s cell histiocytosis. Note the eosinophils. |
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Figure
#12A
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Figure
#12B
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Figure 12A and
12B show AP and lateral radiographs of the lumbar spine with flattened
L3 and L5 vertebrae. |
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Figure
#13
MRI of langerhan’s
cell histiocytosis in the spine with vertebrae planae at L3 and L5
at the arrows. |
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Treatment of this disease is conservative and allows the disease to
burn out. If the pain is unbearable,
then curettage and steroid injection can be helpful. As a last resort, low-dose XRT for lesions in the spine or pelvis
can also be helpful.
LCH has a peculiar association with Diabetes Insipidus that combine to
create Hans-Christian-Schueller Disease
that will show extreme thirst, polyuria,
and vertebrae planae.
