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University of Alberta Walter C. Mackenzie Health Sciences Centre Edmonton, Alberta T6G 2B7, Canada
A 20-year-old man was admitted to our hospital after presenting with shortness of breath that was progressive over a 2-month period. Findings at physical examination included stridor and basilar crepitations. Approximately 2 years earlier, the patient had presented with a 4-month history of diffuse yellowish brown skin lesions, polyuria, polydipsia, visual disturbances, and ataxia. MR imaging of the brain at that time showed multiple lesions involving the brain and the right optic nerve, and pathologic analysis of the skin lesions confirmed a diagnosis of xanthoma disseminatum.
Chest radiographs at admission showed symmetric narrowing of the subglottic trachea and both the right and left proximal mainstem bronchi (Fig. 1A). Unenhanced CT of the thorax confirmed smooth concentric thickening of the subglottic trachea and central bronchial walls, the latter measuring 5 mm (Figs. 1B and 1C). The trachea was narrowed to a diameter of 8 mm.
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Bronchoscopy performed 2 weeks after the chest CT revealed stenosis of 6 mm in diameter extending from 1.0 cm below the glottis for a distance of 1.5 cm. After failure of conservative steroid treatment, the patient underwent tracheal dilation and cold knife dissection. One month later, the patient returned with progressive shortness of breath. Repeated bronchoscopy showed no recurrent tracheal narrowing, but significant stenosis of the left and right mainstem bronchi had developed; the bronchi were narrowed to 2–3 mm and 3–4 mm, respectively. Dilation of the right and left mainstem bronchi was performed with a rigid bronchoscope, with resulting symptomatic improvement. Biopsy confirmed mucosal infiltration of xanthoma disseminatum.
Xanthoma disseminatum, otherwise known as non–Langerhans cell histiocytosis [1], is a benign, nonfamilial mucocutaneous disorder. This rare disorder (only 100 cases have been reported [2, 3]) can occur in either sex at any age but typically involves male children and young adults (male:female ratio, 2:1) [1, 2]. The pathogenesis is unclear; xanthoma formation and deposition are triggered by an unknown stimulus [1]. The histopathology of this disorder helps differentiate it from the more common Langerhans cell histiocytosis. Scalloped histiocytes predominate in xanthoma disseminatum, and the Langerhans' histiocyte that predominates in the disease that bears its name is not present. Skin lesions are common, and mucous involvement is seen in 40–50% of patients, primarily involving the larynx, pharynx, mouth, trachea, epiglottis, and tongue [1]. Circumferential upper airway narrowing due to xanthoma deposition can cause respiratory distress, warranting surgical intervention. Lower respiratory tract involvement, as seen in our patient, is rare, with only three cases reported in the literature [3]. Neurologic symptoms can result from infiltration of the central nervous system. Infiltration of the hypothalamus, as occurred in our patient, results in diabetes insipidus.
Upper and lower airway stenosis can result from one of many and varied causes. One of the most common causes of narrowing is postintubation stenosis, which usually affects the trachea above the thoracic inlet [4]. A saber sheath trachea, goiter, thyroiditis, and thyroid and tracheal neoplasms should also be considered as potential causes. Less common causes of tracheal stenosis include conditions such as idiopathic focal cervical stenosis, relapsing polychondritis, tracheobronchopathia, osteochondroplastica tracheobronchial amyloidosis, Wegener's granulomatosis, sarcoidosis, ulcerative colitis, laryngotracheal papillomatosis, and tracheomalacia [4].
In summary, xanthoma disseminatum is an exceedingly rare benign idiopathic disorder of unknown cause that results in deposition of xanthomas in multiple tissues, including the airway mucosa, which, in our patient, resulted in significant morbidity. Although uncommon, xanthoma disseminatum should be considered in the differential diagnosis of upper and lower airway narrowing when the clinical setting is appropriate.
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