Lepromatous leprosy with laryngeal involvement

Sir,

Leprosy,caused by Mycobacterium leprae, classically affects the skin and peripheral nerves, while involvement of the testes, eyes, lymph node, liver, spleen, bone, bone marrow, muscle and larynx is uncommon. Laryngeal involvement presents with cough, hoarseness of voice, dyspnea and rarely, life-threatening airway obstruction.¹ Implementation of multidrug therapy has further reduced the occurrence of airway obstruction due to laryngeal leprosy, except a few sporadic cases.² In the Philippines, newly diagnosed leprosy cases reached 1908 in 2017, almost 90% being multibacillary. Among them 1.9% had grade 2 disabilities at presentation, thus suggesting a relatively high rate of delayed detection.³

A 48-year-old Filipino male with longstanding lepromatous skin lesions presented with progressive hoarseness and dysphonia, subsequently followed by difficulty in breathing. The patient had been delaying treatment due to economic constraints and finally approached us with worsening symptoms. Cutaneous examination demonstrated leonine facies, madarosis, saddle nose deformity, nodules and ulcerations on bilateral ears and deformities affecting his feet [Figure 1]. Monofilament test revealed impaired sensation over all digits, although no peripheral nerve was enlarged. Motor test, laboratory tests and chest X-ray were unremarkable. Skin biopsy and Fite-Faraco stain confirmed lepromatous leprosy [Figure 2].

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Figure 1a:

Leonine facies, madarosis and saddle nose deformity

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Figure 1b:

Grade 2 deformities affecting bilateral feet.

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Figure 2a:

Atrophic epidermis and nodular perivascular, periadnexal and perineural infiltrates on histology (H&E, ×40)

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Figure 2b:

Fite-Faraco stain demonstrating a bacillary index of 6+ (FiteFaraco, ×400)

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Indirect laryngoscopy demonstrated a fungating epiglottic mass, enlarged arytenoids and markedly narrowed glottic opening due to thickened vocal cords [Figures 3a and 3b]. We observed a friable fungating mass extending from the arytenoids to the true vocal folds on direct laryngoscopy. [Figure 3c]. A computerized tomography scan of the neck demonstrated narrowed glottic opening due to thickened epiglottis, aryepiglottic folds and false vocal cords [Figure 3d]. Histopathology of the arytenoid mass with Fite-Faraco stain showed numerous acid fast bacilli [Figure 3e], confirming the diagnosis of lepromatous leprosy associated laryngeal involvement. Emergency tracheostomy was performed by the otorhinolaryngology surgeons. Post-procedure, we started multidrug therapy for multibacillary leprosy. While on his 2^nd blister pack, the patient underwent fiberoptic endoscopic evaluation to detect lack of vocal cord mobility and impaired swallowing.

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Figure 3a:

Indirect laryngoscopy demonstrated a solitary fungating mass on the epiglottis

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Figure 3b:

Bilaterally enlarged arytenoids and markedly narrowed glottic opening

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Figure 3c:

Direct laryngoscopy demonstrating a friable fungating mass extending from the arytenoids up to the true vocal folds

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Figure 3d:

Plain CT scan of the neck demonstrating narrowed glottic opening due to thickened epiglottis, aryepiglottic folds and false vocal cords

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Figure 3e:

Numerous AFB noted on the arytenoid mass Fite-Faraco stain (Fite-Faraco, ×400)

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After his completion of 11^th blister pack, we noticed the resolution of ulcers on both ears, without any new skin lesions or sensory or motor deficits. Re-evaluation by the otorhinolaryngology department demonstrated persistent thickening of his epiglottis and vocal folds, thus mandating maintenance tracheostomy. After six months, the otorhinolaryngology department shall reassess the patient to determine feasibility of decannulation.

In all reported cases of leprosy with laryngeal involvement, lepromatous leprosy with widespread skin and cutaneous nerve involvement preceded their laryngeal symptoms. Laryngeal lesions develop insidiously, with an asymptomatic phase for years. Symptoms include hoarseness, dry cough, sore throat, difficulty in breathing, dysphagia, dysphonia, aphonia, odynophagia, raspy breathing, stridor and pain when speaking.^1,4 Laryngeal involvement is confirmed by histopathologic demonstration of acid-fast bacilli.¹

Usually, laryngeal leprosy starts from the epiglottis.^4,5 This occurs as inspired air flows across the posterior choanae over the epiglottis reducing local temperature by ~2°C, thus favouring the growth of Mycobacterium leprae due to its predilection for cooler temperatures. Once the epiglottis is affected, infiltration of other laryngeal structures may occur. Vocal cord infiltration is responsible for hoarseness, due to restricted mobility; vocal cords are usually involved later in the disease process, hence the lag time between cutaneous and laryngeal symptoms.⁴

Long-standing laryngeal lesions may become ulcerative or fibrotic. The former results in early development of granulation tissue with subsequent inflammation and rapid edema, which may lead to acute respiratory distress.⁴ Conversely, in the fibrotic form, gradual fibrous tissue formation occurs, possibly leading to laryngeal stenosis and manifesting as aphonia or rarely an upper airway obstruction requiring tracheostomy.^4,5

Multidrug therapy effectively reduces bacterial count in laryngeal leprosy,⁴ although the prognosis remains unaffected, as evidenced by variable treatment outcomes. While few authors reported normalizations of voice post-treatment, most developed persistent dysphonia. Rarely, emergency tracheostomy is necessary, similar to our patient, due to irreversible destruction and scarring of the larynx.^2,5Although multidrug therapy is effective, the authors hypothesize that fibrotic form of laryngeal leprosy is recalcitrant to treatment, owing to underlying scarring and fibrous tissue deposition.

The present rare case of laryngeal leprosy highlights the importance of laryngeal examination in patients with lepromatous leprosy to prevent complications such as laryngeal stenosis and airway obstruction. Moreover, this case emphasizes the need for health education and community awareness to promote early detection and treatment of this disease to prevent its permanent and life-threatening sequelae.