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Clinical Note |

Laryngeal Involvement in Progressive Nodular Histiocytosis A Case Report

Kian Karimi, MD; Armon Jadidian, MD; Frederick L. Glavin, MD; Hardeep Chhatwal, BDS; Robert T. Adelson, MD
Arch Otolaryngol Head Neck Surg. 2010;136(5):513-516. doi:10.1001/archoto.2010.49.
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Progressive nodular histiocytosis (PNH) belongs to an exceedingly rare family of benign proliferative skin disorders known as non-Langerhans cell histiocytoses (NLCHs). Since its initial description as progressive nodular histiocytoma by Taunton et al1 in 1978, there have been only a handful of case reports describing this rare entity. In the past, there has been a great deal of confusion in classifying the various histiocytoses owing to the rarity in which they were encountered as well as to the tremendous overlap, both clinically and histologically, observed between these diseases.2 The lesions encountered in this family of diseases are generally benign; however, in the case reported herein, the involvement of the larynx led to partial airway obstruction, a clinical occurrence previously undescribed (to our knowledge).

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Figure 1.

Our patient in her mid 20s. Her lesions initially appeared on her back and then worked their way up to her neck and finally to her face.

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Figure 2.

The patient when she presented to our clinic. A, Besides the disfiguring facial lesions, she complained of airway obstruction, which was thought to be mainly from the large bulky lesions occluding the nasal airway. B, Complete occlusion of the nasal airways by large, pedunculated lesions. Note the relatively normal appearance of the nasal mucosa. The rest of the nasal cavity was devoid of lesions.

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Figure 3.

Axial postcontrast computed tomographic scan of the neck. A, Thickened aryepiglottic folds with prominence of the left side (arrow). B, A subsequent scan obtained several months later showing slight interval thickening in the glottic region.

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Figure 4.

Laryngoscopic views. A, Marked thickening of epiglottis (arrow) is evident. The epiglottis was noted to be quite firm. The vallecula is shown (arrowhead). B, View of the posterior glottis. Note the thickened arytenoids, on the left greater than on the right.

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