Histiocytosis X, or Langerhans cell histiocytosis (LCH), is a rare disease that consists of three less distinct, overlapping states: eosinophilic granuloma (EG), Hand-Schüller-Christian (HSC) disease, and LettererSiwe (LS) disease. Of the three, EG is the least severe and most localized form, and has the best prognosis. Temporal bone presentation is clinically similar to acute otomastoiditis. W...

An 8 year old boy who had Langerhans cell histiocytosis when he was 15 months old showed psychomotor regression from the age of 2 years. Microcephaly, severe growth deficiency, and ocular telangiectasia were also evident. Magnetic nuclear resonance imaging showed cerebellar atrophy. Alphafetoprotein was increased. Chromosome instability after x irradiation and rearrangements involving chromosom...

The authors present a rare case of C-6 vertebral involvement in a 12-year-old boy with histiocytosis X. The patient presented with limitation in movements of neck and upper extremities. Computed tomography (CT) and magnetic resonance (MRI) imaging were used in the preoperative workup. Surgery was performed via an anterior cervical approach along with stabilization using a fibula strut graft and...

Resolution rate and qualitative characteristics of healing were studied in 71 osseous lesions of histiocytosis X. Rates of healing, as assessed planimetrically and by vertebral body-height measurement, did not differ significantly among lesions that were treated with chemotherapy alone, chemotherapy plus radiation, radiation alone, or those that received no treatment. Lesions of Letterer-Siwe d...

This study describes the chest radiographs of 50 adult patients with histologically verified histiocytosis X, proposes a radiological classification, and examines the role of radiology in assessing the prognosis of the disease. Radiologically the lesions predominate in the middle and lower lung fields, usually sparing the costophrenic angles, and are typically micronodular, reticular, or cystic...

Figure 1: Gastric endoscopy. Numerous polyps cover the entire wall ofthe stomach. Abstract A rare case of gastric polyposis caused by infiltration of Langerhans' celis is reported. A 53 year old Japanese woman complaining of vague abdominal discomfort, was found at endoscopy to have numerous polyps all over the gastric wall. An endoscopic biopsy specimen showed characteristic infiltration of La...