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Case of the Month

April 2019

Dr D Steyn, Dr S Potgieter, Dr E Glover
Universitas Academic Hospital

Case Presentation:
A 56yr old female from Bloemfontein was referred to the Department of ENT at Universitas Academic Hospital in November 2018 with a 6 month history of dysphagia, odynophagia, dysphonia and otalgia. Upon presentation she was completely unable to swallow solids and semi-solids.

She had been started on empirical TB treatment in February of the same year after seeking medical attention at a local hospital for marked weight loss and generalized lymphadenopathy. Sputum at that stage was TB geneXpert negative but biopsy of a supraclavicular lymph node showed granulomatous inflammation with caseous necrosis and a histological diagnosis of TB was thought compatible. Unfortunately no tissue samples were sent to the TB laboratory for microbiological confirmation. Despite 9 months of TB treatment she had progressive weight loss, dropping 4 dress sizes. She had no known chronic diseases and was on no chronic medication. She had no significant surgical history and had never smoked or used alcohol. She was born in a rural village in the Eastern Cape and had spent her most of her life there. Her family had moved her to Bloemfontein in the previous year when she had become too unwell to stay alone. She had no travel history and no occupational exposure

Upon examination she was noted to be cachectic with conjunctival pallor. The previously reported generalized lymphadenopathy was not present. She had hoarseness of voice and stridor. She was not able to swallow even saliva due to a large mass at the base of her tongue. Apart from the stridor, cardiopulmonary exam was normal as was abdominal examination.

Upon laryngoscopy she was found to have a large mass lesion extending from the right tongue base to involve the epiglottis, posterior pharyngeal wall and larynx. A CT scan of her head and neck was requested and is shown below.

Blood workup showed a decreased Hb with red cell indices in keeping with anaemia of chronic disease. Marked hypokalaemia and hypoalbuminaemia was present and thought to be due to malnutrition associated with long standing difficulty in swallowing solids. UEC and LFT were further normal. HIV ELISA was negative. The patient was admitted to the ward and prepared for theatre the following day where she underwent biopsy of the lesion and tracheostomy insertion for imminent airway obstruction.

Based on the findings at laryngoscopy as well as the CT features the only differential diagnosis considered was a base of tongue malignancy.

Biopsy samples were sent for histological and microbiological examination. Initial cultures grew only oral flora with negative mycobacterial and fungal cultures. Histology of the lesion was reported to be in keeping with Rhinoscleroma. Mycobacterial and fungal stains were negative as was tissue TB geneXpert. No evidence of malignancy was found. We requested a repeat biopsy, which again excluded malignancy, mycobacterial and fungal infection and was reported as being histologically in keeping with Rhinoscleroma.

Question 1: What is Rhinoscleroma?

Answer to Question 1

Rhinoscleroma is a rare chronic, slowly progressive infectious disease caused by the inhalation of the gram-negative bacterium Klebsiella rhinscleromatis The organism is endemic in the tropical to temperate zones of Africa, Asia, Eastern Europe and South and Central America, especially in poorer socio-economic settings. Many authors favor the term Scleroma over Rhinoscleroma as although the disease process usually involves the nasal cavity (95% of patients) it can also involve the nasopharynx (up to 43%) oropharynx (13 -35%), larynx (15-40%) and trachea (12%) and bronchi (2-7%). It usually occurs at the transitional regions between two types of epithelium (squamous, ciliary, and respiratory). Risk factors such as crowded conditions, poor nutrition and poor hygiene appear to play an important role in the pathophysiology of the disease but in all other regards patients are immunocompetent.

Question 2: What is the clinical presentation of Rhinoscleroma

Answer to Question 2


Rhinoscleroma most often affects adults in the 2nd -4rd decades of life. Our patient was thus older than that typically described. For unclear reasons, female predominance is seen. There are 3 distinct clinical stages seen. The rhinitic or exudative stage is characterized by a foul smelling mucopurulent discharge with nasal obstruction and oedema. After months to years progression of disease is seen and a florid or proliferative stage is described marked by mucosal thickening, mass lesions and obstruction. The final stage is known as the fibrotic stage and is characterized by scaring and stenosis.

Question 3: How is the diagnosis of Scleroma confirmed?

Answer to Question 3


The diagnosis is confirmed with biopsy (usually during the proliferative stage). Histologically the presence of Mikulicz cells is diagnostic for Scleroma. Mikulicz cells are large macrophages with abundant foamy cytoplasm that contain the K Rhinoscleromatis bacilli. The bacteria are best seen with the Warthin-Starry stain - this was positive in our patient. Culture of Klebsiella rhinoscleromitis on MacConkey agar is diagnostic but the yield is low – cultures being positive in only about 50% of patients. We were not able to culture the organism in our patient.

Question 4: What is the differential diagnosis of Rhinoscleroma?

Answer to Question 4


Infectious granulomatous diseases may include tuberculosis, actinomycosis, syphilis, leprosy, histoplasmosis, sporotrichosis, zygomycosis, blastomycosis, paracoccidoidomycosis and mucocutaneous leishmaniasis. Noninfectious granulomatous diseases include sarcoidosis and granulomatosis with polyangiitis (Wegener granulomatosis). Lethal midline granuloma and other neoplastic conditions need also be considered.

Figure 1: High power H&E stain showing Mikulicz cells – large macrophages with abundant vacuolated foamy cytoplasm

Figure 2: Gram stain: Mikulicz cell containing gram negative bacilli

Figure 3: Warthin-Starry Stain showing Klebsiella rhinosleromatis bacilli within the Mikulicz cell

Question 5: How is Scleroma treated?

Answer to Question 5


Rhinoscleroma can be managed effectively with a combination of antibiotics and surgical debridement and repair. Numerous antibacterial agents have been described in the management of Rhinoscleroma, usually used in combination. These include streptomycin, tetracyclines, rifampicin, trimethoprim-sulfamethoxazole, and ciprofloxacin. Relapse rate is high and depending on the stage prolonged treatment is necessary (3 -12 months depending on response)

Continue to outcome of case

Back to our patient:
Whilst repeat biopsy results were pending the patient was discharged from hospital with a tracheostomy. The ENT surgeons had discussed the likely diagnosis of advanced cancer with her. Thinking that her prognosis was poor her family had decided to take her back to her home village and had discussed end of life plans. When the diagnosis of Scleroma was once again suggested at histology we called her back to our OPD. She was started on oral trimethoprim-sulfamethoxazole and ciprofloxacin and followed up monthly. After 1 month of treatment she was able to swallow semi-solids with ease. A month later she was tolerating all food. At 3 months repeat laryngoscopy showed complete macroscopic resolution of the lesion and her tracheostomy was removed. She had gained 6kgs. She remains on treatment. The intention is to stop treatment at 6 months and then to follow up closely for relapse.

Take home message:
Rhinoscleroma is a rare disease but the incidence is increasing with cross boarder migration. The clinical presentation can resemble many other diseases and the correct diagnosis often elude doctors for years which might result in significant morbidity and even death due to airway obstruction.

References:

  1. Zamora JG, Murali AR. Rhinocleroma with pharyngeal involvement caused by Klebsiella ozaenae. Case Rep Infect Dis. 2016; 2016: 6536275.
  2. Botelho-Nevers E, Gouriet F, Lepidi H, et al. Chronic nasal infection caused by Klebsiella rhinoscleromatis or Klebsiella ozaenae: two forgotten infectious diseases. Int J Infect Dis. 2007;11(5):423-29.
  3. Chan TV, Spegel JH. Klebsiella rhinoscleromatis of the membranous nasal septum. J Laryngol Otol. 2007;121(10):998-1002.
  4. De Pontual L, Ovetchkine P, Rodriguez D, et al. Rhinoscleroma: a French national retrospective study of epidemiological and clinical features. Clin Infect Dis. 2008;47(11):1396-402.

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