1
Introduction
Rhinoscleroma is a chronic granulomatous, slowly progressive infection that affects the nose and other respiratory tract structures. It was first described in 1870 by dermatologist Ferdinando Von Hebra and posteriorly named respiratory scleroma, which emphasizes involvement of upper and lower airways . It occurs frequently in the nasal fossae, eventually extending itself to the larynx, the rhinopharynx, the mouth and the paranasal sinuses; the lips, trachea, and bronchi may also be affected to a lesser degree. Extrarespiratory involvement has rarely been described. Scleroma is endemic in some Central American countries, Indonesia, India, Poland, Hungary, Russia, and some African countries. In China the frequency of this disease is low. The regions of endemicity is Shandong province. The infection is due to a capsulate gram-negative bacterium, Klebsiella rhinoscleromatis , which was first described by Von Frisch in 1882 .
2
Case report
In December 2007, a 73-year-old married male patient who was a native of Beijing was brought to otolaryngology ward at the Beijing Tongren Hospital by his son and presented a mass extruding through the left nasal vestibule ( Fig. 1 ), with progressive bilateral nasal obstruction for ten years. He also had a long history of baryodmia, no sneezing, no nasal pruritus or yellowish rhinorrhea, no daily intense frontal headaches. The specimen was referred to pathology for examination in other hospital and suspected “rhinoscleroma” On examination with the laryngoscopic mirror, left nasal fossae were not pervious. A microlaryngoscopic exploration was performed and showed an enlarged nose due to a wax yellow-colored mass with small ulcers, a hardened consistency, asperate surface, and irregular borders, which extruded from the left nare. There was a similar mass in the back of nasal septum in the right nasal cavity. Computed tomography of the paranasal sinuses showed soft tissue attenuation material occupies the nasal fosse, pachymucosa of bilateral frontal sinus, and submucous cyst of the left maxillary sinus. The cartilaginous septum is deviated to the right. There is no bone destruction. The specimen was referred to pathology for examination. Histopathologic features revealed nasal mucosa containing a diffuse lymphplasmacytic inflammatory process with large vacuolated macrophages typical of Mikulicz cells and many plasmocytes transformed into Russel bodies. The patient was treated with antibiotic therapy. Cefradine was used (500 mg, 12/12 hours for 1 month) based on literature data.
Three months later, the patient again consulted for progressive bilateral nasal obstruction, cough and thick phlegm. A microlaryngoscopic examination revealed the scar of the front rhinoscleroma and the spreading inflammation of the nasal mucosa were persistent. A nodulous mass through bilateral nasal vestibule. Treatment included surgery followed by antibiotics. Surgical removal of the tumor from the nasal vestibule. The specimen was referred to pathology for examination. The second histopathological examination showed an inflammatory reorganization, including plasmocytes, lymphocytes, and giant histiocytes. The patient accepted surgical therapy and the tumor was resected with a safety margin. In order to provide cover of the wound, a free skin flap of abdominal part was designed.
The flap measured 3 cm (in width) by 5 cm (in length) and was raised beginning at its lateral end. Depending on degrees of skin redundancy and elasticity, the flap was extended longitudinally as necessary to optimize the length-to-width ratio that would allow for primary closure without undue tension. The full-thickness skin graft was harvested with a number 10 blade and defatted using sharp scissors. The skin graft was sutured peripherally to the nasal vestibule defect. Postoperatively no complication was noted and the flap survived. The patient returned to the otorhinolaryngology unit last week was in good physical condition and no signs of recurrence in the nasal mucosa ( Figs. 2 and 3 ).
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