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Allergy and Airway |

Diffuse Tracheobronchial Inflammation and Stenosis in Ichthyosis Vulgaris

Harry Nima-Zegarra, MD; Ray Shepherd, MD; Samira Shojaee, MD
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Virginia Commonwealth University Health System, Richmond, VA


Chest. 2013;144(4_MeetingAbstracts):31A. doi:10.1378/chest.1703126
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Abstract

SESSION TITLE: Bronchology Cases I

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Sunday, October 27, 2013 at 01:15 PM - 02:45 PM

INTRODUCTION: Ichthyosis refers to a wide range of keratinizing disorders with different etiologies. Scaling of the skin and thickening of the cornified layer characterize this condition. The two most common forms of ichthyosis, autosomal dominant ichthyosis and X-linked recessive ichthyosis, occur at frequencies of about 1/300 and 1/2500, respectively. A more heterogeneous and rare group of patients also have non-cutaneous symptoms affecting the central nervous system, immune system, skeleton or other non-cutaneous tissues. We present a case of severe tracheobronchial inflammation and stenosis in a patient with congenital ichthyosis.

CASE PRESENTATION: A 25-year-old white male with history of GERD, bronchiectasis, recurrent pneumonias and congenital ichthyosis was referred to our clinic for management of chronic productive cough. His extensive evaluation was non significant and his chest CT scan revealed mild bilateral bronchiectasis that had not changed over many years. He complained of worsening chronic productive cough with moderate amounts of yellowish mucoid sputum and occasional scant amounts of blood. After multiple failures in treatment, flexible bronchoscopy was planned to rule out atypical infections and intraluminal pathology. It was remarkable for an edematous larynx, significant inflammation of the tracheobronchial tree with significant bronchial stenosis at multiple segmental and subsegmental bronchi bilaterally. Trachea and carina forceps biopsy was performed which showed chronic inflammation of the mucosa and lamina propia. Focal areas of squamous metaplasia were identified too. In a subsequent bronchoscopy, balloon dilation was performed in multiple bronchial segments. Immediately following balloon dilation, airway diameter was visibly increased in all segments.

DISCUSSION: This case demonstrates a unique presentation of an adult patient with a diagnosis of congenital ichthyosis vulgaris involving the airways. The extensive airway involvement leads to mucosal inflammation, stenosis and partial obstruction, which most likely are responsible for this patient’s symptoms. Congenital ichthyosis in its most severe presentations has been associated with pulmonary complications in neonates and children. Neonatal asphyxia/ARDS and pneumonias are the most common respiratory complications at early age. Postmortem examination has revealed keratin debris filling the bronchial tree and alveoli 1. This has not been reported in the adult population.

CONCLUSIONS: Tracheobronchial inflammation and stenosis with Ichthyosis vulgaris, is not reported in the literature. Bronchoscopy and balloon dilation may play a roll in the management of airway stenosis in this setting.

Reference #1: Khnykin D, Ronnevig J, et al. Ichthyosis Prematurity Syndrome: Clinical Evaluation of 17 Families with a Rare Disorder of lipid metabolism. J Am Acad Dermatol 2012; 66 (4): 606-16

DISCLOSURE: The following authors have nothing to disclose: Harry Nima-Zegarra, Ray Shepherd, Samira Shojaee

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