A Case of Nasal Cavity and Laryngeal Involvement of Rosai–Dorfman Disease

Rosai–Dorfman disease (RDD) is a rare non-malignant disorder, characterized by painless multiple cervical lymphadenopathy, fever, and elevated inflammatory markers. Its diagnosis is difficult due to its rare incidence and various clinical presentations, especially in extranodal involvement. In this report, we demonstrate a patient with RDD who presented with a nasal septum and laryngeal tumor that caused dyspnea. We achieved a successful treatment outcome with combined surgical resection of the laryngeal mass and corticosteroid medication. The symptoms and tumors were resolved within 3 weeks after treatment. We reported our experiences with review of literatures.

Characteristics and Clinical Outcome of 295 Patients with Relapsing Polychondritis

Objective This study analyzes the clinical features of Chinese patients with relapsing polychondritis (RP). Methods The clinical data of 295 patients with RP of Beijing Tongren Hospital were retrospectively analyzed. Results The mean age of onset was 41.0 ± 15.0 years. The sex ratio was 1:1. Up to 70.5% of the patients had airway involvement during the disease course; among them, the larynx was most commonly affected (82.2%). Exactly 25.7% of the patients with laryngeal involvement underwent tracheotomy due to progressive dyspnea or acute laryngeal obstruction. Younger onset age and initially presenting with respiratory symptoms were independent risk factors for tracheotomy in patients with RP with laryngeal involvement. The risk of tracheotomy in patients who presented with respiratory symptoms was 2.354 times higher than that in patients who presented with other symptoms (hazard ratio [HR], 2.354; 95% confidence interval [CI], 1.230–4.503; p =0.010). The risk of tracheotomy increased by 4.8% for every 1 year decrease in the onset age (HR, 0.952; 95% CI, 0.931–0.973; p < 0.001). The incidence of lower respiratory tract infection was much higher in patients with airway involvement than that in those without airway involvement. The main cause of death was respiratory failure due to airway obstruction. Conclusion There is a high prevalence of airway involvement in Chinese patients with RP. Laryngeal involvement is associated with a high risk of death. More attention should be paid to RP patients with laryngeal involvement who are young at disease onset and present with respiratory symptoms.

A RARE CASE OF NEUROFIBROMATOSIS WITH SQUAMOUS CELL CARCINOMA OF EPIGLOTTIS

INTRODUCTION: The neurofibroma is a nonmalignant new growth of neuroectodermal origin. neurofibromas appear at the end of a nerve, often in the skin, producing small nonencapsulated nodules which may have pigmentation in the overlying skin. Neurofibromatosis with laryngeal involvement presents with dyspnea, followed by hoarseness, stridor, dysphagia, and voice change. most common sites involved in the larynx are the arytenoids and the aryepiglottic folds. CASE REPORT: 65-year-old female with previously diagnosed of neurofibromatosis since 10 years of age manifested by multiple cutaneous nodules The patient had symptoms of dysphagia, hoarseness of voice for past 2 months. Direct laryngeal examination using flexible endoscope showed a edematousulceroproliferative growth in the laryngeal surface of epiglottis and fullness in the left pyriform fossa. Hisopathological examination revealed squamous cell carcinoma from epiglottis and fibromatosis changes from pyriform fossa. CONCLUSION: All neurofibromatosis patient and their family members should under go regular oral and laryngeal examination to rule out complications at the earliest.

A Sweet Voice: Acute Febrile Neutrophilic Dermatosis of the Larynx

Significance Statement Acute febrile neutrophilic dermatosis (Sweet syndrome) is a rare idiopathic condition characterized by fever and whole-body rash of tender erythematous plaques of unknown etiology. Otorhinolaryngologic manifestations of the disease can be severe, yet they are sparsely reported in the literature. We present the first documented case of laryngeal involvement of Sweet syndrome.

Respiratory Distress Complicating Laryngeal Sarcoidosis

Laryngeal involvement in sarcoidosis is rare but can be potentially dangerous. A Severe obstruction of the laryngeal tract may appear, sometimes requiring a tracheostomy. Systemic corticosteroid therapy is the treatment of choice for most patients. We report the case of a 25-year-old patient with isolated laryngeal sarcoidosis for 12 years. She was admitted for respiratory distress. The nasofibroscopy revealed diffuse supraglottic edema, the medical care consisted of an intense surveillance and a corticosteroid therapy. Isolated laryngeal sarcoidosis is a condition that requires multidisciplinary consultation. It presents a challenge in terms of diagnosis, in terms of appropriate management of the respiratory tracts and in terms of treatment.