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Pathology of Langerhans Cell Histiocytosis of the Ear

 Dr Sampurna Roy MD    


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A high index of suspicion is required to recognize the otologic manifestations of histiocytosis X for two reasons:

1) the systemic manifestations of the disease are often so dramatic that the ear findings are overlooked, and

2) the otologic findings of histiocytosis X can mimic more common diseases, including simple otitis externa, aural polyps, acute mastoiditis, chronic otitis media, and metastatic lesions.

Langerhans cell histiocytosis (LCH) is a rare condition of children and young adults in which Langerhans cells proliferate.

The clinical spectrum ranges from solitary or few focal lesions to multisystem involvement mimicking vasculitis or hematological malignancy.

Histiocytosis X or Langerhans cell histiocytosis (LCH) is a disease that possesses three less distinctive and overlapping states called eosinophilic granuloma (EG), Hand-Schuller-Christian (HSC) disease and Letterer-Siwe (LS) disease.

Facial bone involvement may manifest as an ear discharge, hearing loss, or exophthalmos.

EG is the least severe and localized form of all LCHs and possesses the best prognostic result. A high index of suspicion is required to diagnose the EG, especially when an ear disease is refractory to medical treatment.

LCH is easy to be misdiagnosed. Children below 2 years old tend to complicate with multiple organ failure and bronchopneumonia which have high death rate.

The temporal bone may be the first site affected, usually in the medial part of the external auditory canal in children. It is a very rare condition.

The middle ear and the mastoid are involved 61% of cases. The inner ear  is more resistant to the destruction caused by the granulation tissue, but when occurs, an irreversible neurosensorial hearing loss appears.

The signs and symptoms of otologic histiocytosis can mimic those of acute and chronic infectious ear disease.

Pathology examination is helpful for early diagnosis, treatment, and prevention of severe complications.

Visit: Paediatric Pathology Online ; Pulmonary Eosinophilic Granuloma ; Langerhans cell histiocytosis (Histiocytosis X).

Further reading:

Radiculopathy as a manifestation of Langerhans' cell histiocytosis.

Cochlear implantation for treatment-induced ototoxic deafness in Langerhans cell histiocytosis. A case report.

Mondini dysplasia and recurrent bacterial meningitis in a girl with relapsing Langerhans cell histiocytosis.

Langerhans' cell histiocytosis of the labyrinth in adults.

Langerhans' cell histiocytosis with bilateral temporal bone involvement.

Eosinophilic granuloma of the temporal bone.

Ear involvement in childhood Langerhans' cell histiocytosis. 

Langerhans' cell histiocytosis--a rare cause of sudden onset unilateral sensorineural hearing loss.

A case of bilateral eosinophilic granuloma in the temporal bone. 

Labyrinthine involvement in Langerhans' cell histiocytosis.

Aural symptoms as primary presentation of Langerhan's cell histiocytosis.

Unifocal Langerhans' cell histiocytosis (eosinophilic granuloma) of the petrous apex.

Eosinophilic granuloma of the ear. General review apropos of 2 case reports

Histiocytosis X of the ear and temporal bone: review of 22 cases.

Otologic manifestations of Langerhans' cell histiocytosis

Labyrinth involvement in Langerhan's cell histiocytosis.

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Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)







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