Thursday, August 21, 2014

Scleroma - a nasty infection of the respiratory tract

Nasal inflammation, thickened mucosa, ulcers and polyp-like masses that form within the nose and throat, ultimately leading to airway obstruction, scarring and deformity - chances are you've never heard about this ugly malady before.

Scleroma is a chronic disease that affects the upper respiratory tract. It used to be endemic in eastern Europe; now it occurs mostly in tropical regions, such as North Africa, India, South America and Central America. The causative organism is a Gram-negative bacterium, Klebsiella rhinoscleromatis.

The bacteria that cause this highly unpleasant condition can be transmitted by directly inhaling infected droplets or by contact with contaminated material. Undernourishment and a lack of hygiene make it easier for the infection to take hold. It usually begins at the nose and may spread to the pharynx, larynx, trachea or - rarely - even the bronchi. The sinuses and soft palate may be involved, too. The nose is affected in 95-100% of cases (scleroma which affects the nose and no other parts of the respiratory tract is called rhinoscleroma). For some reason, nasal and pharyngeal scleroma is more common in males (male-to-female ratio, 2:1), whereas laryngotracheal scleroma is more common in females (female-to-male ratio, 4:1). 

Scleroma tends to progress slowly over many years, and is characterised by periods of remission and relapse. Initially, patients show symptoms of rhinitis, which may include an odorous, purulent discharge, crusting and various degrees of nasal obstruction. This early stage of the disease is known as the catarrhal stage. As the infection progresses to the granulomatous stage, well-defined masses of granulation tissue - rubbery polyps - typically develop in the affected regions. These masses can actually be mistaken for a nasal tumor. In this stage of the disease, epistaxis (bleeding from the nose) is common, and patients often lose their sense of smell (anosmia). Destruction of the nasal cartilage may occur. Frequently, the nose becomes significantly deformed; in some cases, the nasal septum is completely destroyed. Advanced scleroma causes scarring and fibrosis of the affected tissues. Altogether, the symptoms of this disease bear some similarity to leprosy, although the causative organism is completely different.

Scleroma is difficult to cure and prone to recur. Currently, the recommended treatment consists of a combination of surgical debridement and long-term antibiotic therapy. However, in tropical regions where this disease is endemic, medical care often isn't easily accessible or affordable for the sufferers. In non-endemic regions such as the United States, scleroma used to be rare, but cases occur more frequently now, due to increased migration, and aren't always diagnosed swiftly enough.

Not nice, is it? And here are some pictures:

Pharyngeal scleroma. A CT scan of the oropharynx shows narrowing of the oropharyngeal airway with abscesses in both tonsillar regions and V-shaped calcification along the soft palate.  
(Image from: Razek 2012)

A 45-year-old lady with septal rhinoscleroma, presenting as a large swelling on one side of the nose. (Image from: Shoeib 2010)

An article by Shoeib (2010) describes the case of a female patient aged 45 years who presented with a large swelling on her nose (see picture above) which had been present since 1 year. There was past history of nasal trauma. The nose was swollen and obviously asymmetric, with unilateral obstruction, but no other symptoms such as bleeding or crusting. Internal nasal examination showed a septal swelling protruding to the right nasal cavity, with mucosal hypertrophy. The mass was surgically excised, and the resulting defect was reconstructed with cartilage grafts. Histopathological examination of the specimen revealed the cause - rhinoscleroma. Postoperatively, the patient received antibiotic treatment in the form of ciprofloxacin. The functional and aesthetic outcome was good, with no recurrence after 6 months.

Gupta and Agrawal (1998) have described an unusual case where scleroma manifested as a solitary nodule on the cheek of a 66-year-old man from an urban slum in India. It was treated by surgical excision and antibiotics.

Chan TV, Spiegel JH. (2007) Klebsiella rhinoscleromatis of the membranous nasal septum. J Laryngol Otol. 121(10): 9981002. Epub 2007 Mar 15.

Gupta A, Agrawal SR (1998) An unusual case of solitary scleroma of cheek. Indian J Otolaryngol Head Neck Surg. 50(3): 296297.

Razek A. (2012) Imaging of scleroma in the head and neck. Br J Radiol. 85(1020): 1551–1555.

Shoeib MA. Septal rhinoscleroma. Indian J Plast Surg. 43(2): 219–221. 

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