Langerhans cell histiocytosis

Case contributed by Dr Ruslan Asadov
Diagnosis probable

Presentation

Diabetes insipidus, cough, fever, shortness of breath.

Patient Data

Age: 20 years
Gender: Male
This study is a stack
Axial
non-contrast
This study is a stack
Axial bone
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Info

Lytic defect of the parietal, frontal and maxillary bones in the skull.

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Conventional CT reveals multiple small nodules and thin-walled, irregular cysts predominantly in the upper 1/3 of the lungs.

Case Discussion

Langerhans cell histiocytosis. The hand-Schüller-Christian disease is the chronic disseminated form, multifocal.

Other lytic lesions are identified in the femur (no image).

Etiology: Uncertain; Inflammatory ~ neoplastic

Epidemiology:  Affects 4 per 1 million                                                             

 Gender: M:F = 2:1

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