eMedicine Specialties > Dermatology > Bacterial Infections

Rhinoscleroma: Differential Diagnoses & Workup

Author: Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Coauthor(s): Egle Goriniene, MD, Staff Physician, Department of Infectious Diseases, New Jersey Medical School
Contributor Information and Disclosures

Updated: Jul 2, 2009

Differential Diagnoses

Other Problems to Be Considered

Vasculitis
Neoplastic disease (eg, lymphoma)
Extranodal Rosai-Dorfman disease
Nasal polyposis⁹

Infectious granulomatous processes may include those caused by bacteria (tuberculosis, actinomycosis, syphilis, leprosy), fungi (histoplasmosis, blastomycosis, paracoccidioidomycosis, sporotrichosis), and parasites (mucocutaneous leishmaniasis).

Rhinoscleroma can mimic various inflammatory and neoplastic processes, including leprosy, paracoccidioidomycosis, sarcoidosis, basal cell carcinoma, and Wegener granulomatosis. Rhinoscleroma should be added to the list of opportunistic infections that can occur in patients infected with the human immunodeficiency virus.

Granulomatous lesions of the craniofacial area are common. These lesions vary in nature. They can be lymphohistiocytic with or without eosinophils; they can be tuberculoid with epithelioid cells and giant cells; or, occasionally, they are composed of essentially giant cells. The etiology of these lesions may be known or easy to discern. Their causes include foreign body granulomas, sarcoidosis, leprosy, rhinoscleroma, fungal diseases (especially zygomycosis and rhinosporidiosis¹⁰ ), parasitic diseases, and cocaine-induced midline destructive erosions.¹¹

Lethal midline granuloma is a clinical entity characterized by a necrotic and relentlessly progressive destructive presentation. After a malignant process (especially lymphoid) and Wegener granulomatosis are eliminated from the differentials, the diagnosis is idiopathic midline nonhealing granuloma. Some lesions remain in the facial area, whereas others disseminate as a malignant disease.

Central giant cell granuloma and histiocytosis X (especially eosinophilic granuloma) are 2 other varieties of granuloma that differ from the aforementioned granulomatous infiltrates in their clinical presentation and evolution.

Workup

Laboratory Studies

• Laboratory markers
  • A positive result with culturing in MacConkey agar is diagnostic of rhinoscleroma.
  • However, culture results are positive in only 50-60% of patients.
• Bacterial identification
  • Bacteria can be seen by using periodic acid-Schiff, Giemsa, Gram, and silver stains.
  • A highly sensitive and specific method for identifying K rhinoscleromatis organisms is the analysis of a biopsy specimen with the immunoperoxidase technique.

Imaging Studies

• CT findings in primary nasal and nasopharyngeal rhinoscleroma include soft-tissue masses of variable sizes.
  • The lesions are characteristically homogeneous and nonenhancing, and they have distinct edge definition.
  • Adjacent fascial planes are not invaded.
  • The subglottic area is involved in laryngeal and tracheal scleroma.
  • The lesions primarily cause concentric irregular narrowing of the airway.
  • In the trachea, cryptlike irregularities are diagnostic of scleroma.
  • Findings also include calcifications, luminal stenosis, wall thickening, and nodules.¹²
• MRI should be performed in patients with rhinoscleroma.
  • Nasal masses can obstruct the ostiomeatal units, and secretions may be retained in the related sinuses.
  • In the hypertrophic stage of rhinoscleroma, both T1- and T2-weighted images show characteristic mild-to-marked high signal intensity.

Procedures

• Diagnosis is facilitated by the use of cytologic methods that are easy to perform and do not cause pain in the patient (see Further Outpatient Care).
  • Cytologic analysis is performed on brushing specimens of a lesion.¹³
  • The characteristic cells of the Mikulicz type may be observed in the smear.
• This chronic infectious disease of the upper respiratory tract is routinely diagnosed by means of tissue biopsy of the lesions.
• Nasal endoscopy reveals signs of all 3 stages of scleroma: catarrhal, granulomatous, and sclerotic.
• Bronchoscopy has a role in the early diagnosis of rhinoscleroma.¹⁴

Histologic Findings

Histopathologic analysis has a definite role in the diagnosis of rhinoscleroma. Classic histopathologic findings include large vacuolated Mikulicz cells and transformed plasma cells with Russell bodies. The Mikulicz cell is a large macrophage with clear cytoplasm that contains the bacilli; this cell is specific to the lesions in rhinoscleroma. The disease is most commonly diagnosed during the proliferative phase, in which the clinical and histologic presentations are most easily recognized.

The histologic findings correspond to the 3 clinical stages. In the catarrhal (or atrophic) stage, squamous metaplasia and a nonspecific subepithelial infiltrate of polymorphonuclear leukocytes with granulation tissue are observed. In the granulomatous stage, the diagnostic features include chronic inflammatory cells, Russell bodies, pseudoepitheliomatous hyperplasia, and groups of large vacuolated histiocytes that contain K rhinoscleromatis organisms (Mikulicz cells). If numerous, these bacteria can be seen with hematoxylin and eosin staining, but periodic acid-Schiff, silver impregnation, or immunohistochemical staining may be required to confirm their presence and identity. In the sclerotic stage, extensive fibrosis may lead to stenosis and disfiguration.

Microscopically, the connective tissue is highly vascular, with an inflammatory infiltrate consisting primarily of plasma cells and lymphocytes and a possible sprinkling of eosinophils. Russell bodies in the plasma cells are common. However, the groups, clusters, or sheets of large (100- to 200-μ m) vacuolated histiocytes (ie, Mikulicz cells) that contain the causative agent are most striking. Although the organisms are occasionally visible on standard hematoxylin and eosin stains, they are more readily demonstrated by using silver impregnation Warthin-Starry stains. The exudative stage results in a dense nonspecific fibrosis. In the exudative and cicatricial stages, Mikulicz cells may be difficult to detect.

Electron microscopy reveals large phagosomes filled with bacilli and surrounded by a finely granular or fibrillar material that is arranged in a radial pattern. This finding represents the accumulation of antibodies on the bacterial surface (type A granules), as well as the aggregation of bacterial mucopolysaccharides surrounded by antibodies (type B granules).

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