Lumen narrowing in laryngeal involvement due to progressive nodular histiocytosis

Introduction:

Progressive nodular histiocytosis (PNH) is a very rare form of non-Langerhans cell histiocytosis (non- LCHs). It can be distinguished from other forms by the presence of yellow-red brown papules and large nodules of several centimeters.

Since the first description of PNH by Taunton et al. In 1978, there are only few case reports describing this rare form of disease.

However, in the case reported here, PNH with involvement of the larynx resulted in partial airway obstruction.

Case report:

We report a 39-year-old patient who has been suffering from nodular skin lesions for many years now, which were prominent on his arms and legs.

During intubation for the surgical excision of the skin lesions, the access to the airway was restricted by massive nodular growth in the supraglottic area. We were able to completely resect the lesions with an endoscopic laser resection (CO2 laser).

Histopathology showed a massive infiltration of non-Langerhans cells into the connective tissue, which is most likely of PNH of the type Erdheim-Chester etiology, inspite of the atypical disease presentation.

Conclusion:

Progressive nodular histiocytosis (PNH) is a rare disorder and belongs to a subgroup of non-Langerhans cell histiocytosis (non-LCHs) which is characterized by nodular lesions that usually affect the integument and occasionally the mucous membranes.

Such a disease presented with infiltration of the laryngeal region has a low prevalence. It is Mandatory to consider a preoperative ENT consultation even in asymptomatic patients.

Publication History

Publication Date:
23 April 2019 (online)