Recurrent respiratory papillomatosis (RRP) is the most benign laryngeal neoplasm in children, however it is less common in adults. The causative agent is human papilloma virus (HPV), typically subtypes 6 and 11. The incidence of distal tracheal or pulmonary spread is less than 5%-16%, but morbidity can be high. We present a case of disseminated RRP in an adult male with newly diagnosed sarcoidosis.
A 54-year old diabetic male presented from an outside hospital, after unsuccessful treatment for a diffuse painful lower extremity rash. Routine chest radiograph (FIGURE 1) showed bilateral hilar adenopathy. Computed tomography scan of the chest demonstrated diffuse fine nodule parenchymal infiltrates bilaterally with marked hilar and subcarinal lymphadenopathy. The patient had no fevers, dyspnea, or weight loss. He reported an occasional dry cough. He has tongue “polyps” that have to be removed every few years. He was diagnosed with obstructive sleep apnea about 10 years ago, but has refused to wear the prescribed CPAP at night. He had been on a 2 week course of intravenous antibiotics with piperacillin/tazobactam for presumed cellulitis. A workup for vasculitis was negative. Two days after arriving at our hospital, he developed the same diffuse erythematous painful rash over his elbows. On physical examination, in addition to the rashes, we noted papillomatous lesions on the tongue and verrucae on his hands. No other masses or lymphadenopathy were present. Lungs sounds were unremarkable. Skin biopsy and bronchoscopy for transbronchial needle aspiration (TBNA) of hilar lymph nodes were performed. During bronchoscopy, a large grape-like lesion was encountered 5cm distal to the vocal cords (FIGURE 2). Additionally, we visualized similar, but smaller mucosal lesions distally (FIGURE 3). Endobronchial biopsies revealed squamous metaplasia and inflammation, consistent with RRP. There was no histological evidence of sarcoidosis in the endobronchial tissue. TBNA was also non-diagnostic, so mediastinoscopy was performed and confirmed sarcoidosis with the presence of diffuse mediastinal non-caseating epithelioid granulomas. Skin biopsy also revealed granulomatous inflammation, consistent with cutaneous sarcoidosis. He was started on naproxen for the cutaneous symptoms and had a good response to treatment within four weeks.
While HPV has been defined as the etiologic agent, the true epidemiology of RRP is poorly understood, and management of the lesions is problematic. Rarely, RRP can transform into squamous cell carcinoma. In adults, the typical age at diagnosis is 20 to 40 and the adult form generally is more indolent. Extralaryngeal spread is reported in 16% of adult cases, with oral cavity, trachea, and bronchi being the most common sites, in descending order of frequency. Symptoms of RRP include hoarseness, wheezing, cough, and stridor. Children are often misdiagnosed as having asthma, croup, allergies, or bronchitis because of the symptomatology. Prompt laryngoscopy or bronchoscopy is the key to making a diagnosis. Surgical removal is the mainstay of treatment, with the goal of maintaining a patent airway. Adjuvant medical therapy includes direct intralesional injections of cidofovir subcutaneous interferon, retinoic acids, photodynamic therapy, and ribavirin. Recurrence is common and leads to a high morbidity.
RRP follows an unpredictable course and can result in severe airway compromise. The management is primarily surgical, but direct and systemic medical therapies are adjunctive. To our knowledge, this is the first reported case of simultaneous RRP and sarcoidosis in an adult.
Dominic Valentino III, None.