Vol. 17, No. 4
THE JOHNS HOPKINS MICROBIOLOGY NEWSLETTER
Monday, January 26, 1998
A. Provided by Carmela Groves, R.N., M.S., Chief, Division of Outbreak Investigation, Maryland Department of Health and Mental Hygiene
33 outbreaks were reported between January 12 and January 26, 1998.
Influenza Update: As of January 22nd, there have been 42 lab confirmed cases of influenza A reported in Maryland.
B. The Johns Hopkins Hospital: Information provided by Dr. Blaire Baisden, Department of Pathology
Case Presentation
A 28 year old Mexican male presented to the emergency department with a small ulcer affecting the posterior portion of the nasal septum. The patient was examined and a biopsy was taken. The tissue diagnosis was consistent with rhinoscleroma.
Klebsiella rhinoscleromatis
Klebsiella rhinoscleromatis is the etiologic agent of rhinoscleroma, a chronic progressively inflammatory disease of the upper and rarely, the lower respiratory tract. K. rhinoscleromatis are nonlactose-fermenting, gram negative coccobacilli measuring 2-3 m in length. The organisms are not normally inhabitants of the nasal flora. Most K. rhinoscleromatis strains are capsular type 3.
Rhinoscleroma is endemic to North and Central Africa, Central and South America and Southeast Asia. Sporadic cases have been reported in the United States mainly in persons who have emigrated or traveled to endemic areas. Rhinoscleroma has been identified as an opportunistic infection that can occur in patients with HIV infections following the report of two cases in HIV positive patients who had traveled within an endemic area (1,2).
Pathology
There are three histologic stages of development of rhinoscleroma. In the catarrhal stage, the mucosa contains nonspecific inflammatory changes with neutrophils, cellular debris and granulation tissue. In the proliferative stage, there is an intense infiltrate of plasma cells and large foamy histiocytes termed "Mikulicz cells" which contain numerous rod-shaped bacteria within their cytoplasm. In the last stage, there are variable degrees of fibrosis. Mikulicz cells are characteristically absent.
In the later stages of the disease, lesions undergo fibrosis developing large firm intramucosal nodules which can lead to bony invasion and airway obstruction in severe infections. In patients with fully developed rhinoscleroma, intranasal masses can lead to external expansion of the nose known as "Hebra nose".
The differential diagnosis of rhinoscleroma includes leprosy, sarcoidosis, lymphoma, tuberculosis, mucocutaneous leishmaniasis, various mycotic infections and histiocytosis with massive lymphadenopathy.
Diagnosis
Diagnosis is usually made by the identification of the Mikulicz cells in tissue biopsy. The bacteria may be seen in H&E stained sections, but are more easily identified with PAS, Geimsa or Warthin-Starry stains. Tissue biopsies in the latter stages are often nondiagnostic. Immunoperoxidase stains for Klebsiella capsular antigen III may increase sensitivity (3). K. rhinoscleromatis may also be identified by culture of mucosal lesions.
Therapy
Traditional antimicrobial therapy has included streptomycin, tetracycline and trimethoprim-sulfamethoxazole which may successfully treat early lesions. Prolonged high dose oral ciprofloxacin has been successful in achieving resolution in patients with extensive disease (4). Surgical debridement may be necessary in cases of airway obstruction.
References:
1. Andraca R, Edson RS, Kern EB. Rhinoscleroma: a growing concern in the United States? Mayo Clinic Experience. Mayo Clinic Proceedings 1993; 68 (12): 1151-7.
2. Paul C, Pialoux G, Dupont B, et.al. Infection Due to Klebsiella rhinoscleromatis in Two Patients With Immunodeficiency Virus. Clinical Infectious Diseases 1993; 16: 441-2.
3. Meyer PR, Shum TK, Becker TS, Taylor CR. Scleroma (Rhinoscleroma) A Histologic Imunohistochemical Study With Bacteriologic Correlates. Arch Pathol Lab Med 1983; 107: 377-383.
4. Avery RK, Salman SD, Baker AS. Rhinoscleroma treated with ciprofloxacin: a case report. Laryngoscope 1995; 105: 854-6.