bubble_chart Overview

Scleroma is a chronic progressive granulomatous lesion. It typically begins in the nasal region and slowly extends to the upper lip, nasopharynx, palate, pharynx, larynx, trachea, paranasal sinuses, and nasolacrimal duct. The lesions generally do not invade lymph nodes or metastasize elsewhere. Since over 98% of cases involve nasal lesions, the disease is also referred to as respiratory scleroma or rhinoscleroma. A small number of cases may primarily affect the pharynx, larynx, or trachea without involving the nasal region. Scleroma is a sporadic endemic disease reported worldwide. According to Neijing, statistical analysis of extensive data shows that the disease occurs in all provinces and cities except Taiwan and Tibet, with Shandong Province having the highest incidence rate, accounting for approximately 46% of the total cases.

bubble_chart Etiology

The disease is likely caused by the scleroma bacillus, also known as Frisch's bacillus. In 1882, Frish was the first to discover the pathogen—scleroma bacillus—in the affected tissue. However, subsequent animal and human inoculations often failed, leading many scholars to remain skeptical. The disease has a prolonged course, a long history of exposure, and may be grade I pestilence in nature. Additionally, it is associated with factors such as individual nutritional status, immunity, and environmental hygiene.

bubble_chart Pathological Changes

The pathological changes of scleroma can be divided into three stages: the catarrhal stage, the sclerotic stage, and the cicatricial stage. Different pathological features appear at different stages, but the three may coexist or appear in transitional forms.

bubble_chart Clinical Manifestations

The symptoms depend on the affected site. Common manifestations include stuffy nose, nasal dryness, and epistaxis. If the lesions involve the nasopharynx, eustachian tube, oropharynx, larynx, trachea, or bronchi, corresponding functional impairments may occur, such as tinnitus, ear fullness, hearing impairment, throat dryness and discomfort, dry cough, hoarseness, and dyspnea. Pharyngeal examination reveals varying presentations at different stages of the disease.

(1) Catarrhal stage (also called atrophic or diffuse stage): Early nasal mucosal lesions primarily manifest as dryness, bleeding, and crusting, followed by atrophic changes resembling atrophic rhinitis but without the characteristic foul odor of ozena. The pharyngeal mucosa becomes dry, thin, and shiny, with mucopurulent secretions or crusts adhering to the posterior pharyngeal wall.

(2) Sclerotic stage (also called granulomatous stage): Nodular, tumor-like masses often appear in the nasal vestibule, septum, lower turbinate tip, upper lip, and other areas. These lesions are slightly pale, with a smooth mucosal surface and dilated small blood vessels, and have a hard texture. Beneath the pharyngeal mucosa, scattered nodules of varying sizes—ranging from pinpoint to bean-sized or even forming small patches—may be observed. These nodules feel hard and may be covered with purulent crusts.

(3) Scarring stage: The proliferative sclerotic nodules undergo fibrosis, forming scars and leading to various deformities, such as stenosis or atresia of the nasal vestibule, cicatricial stenosis of the pharynx or larynx, or disappearance of the uvula.

bubble_chart Diagnosis

The clinical diagnosis of typical cases is not difficult. It can be based on the chronic progression of sexually transmitted disease, the location of the lesions, the hardness of nodules and cartilage (pale in color, purplish-red in the advanced stage, generally without ulceration or necrosis but with scar formation, no local pain, and possible coexistence of lesions in the late stage [third stage]), as well as endemic characteristics. A biopsy can confirm the diagnosis. Bacterial culture: grinding nasal secretions, sclerotic tissue, or scar tissue from the atrophic stage into a suspension may reveal scleroma bacilli. Blood moistening and tonifying complement fixation test: Scleroma bacilli can induce antibody production in the patient's serum, making this test highly valuable for diagnosis.

bubble_chart Treatment Measures

The disease course can last 20 to 30 years. While it generally does not affect overall health, isolated cases may die from cachexia or intracranial complications. When scleroma occurs in the pharynx, larynx, or trachea, it can cause upper respiratory tract obstruction, posing a life-threatening risk. Respiratory infections are also prone to occur. During the catarrhal stage of the disease, medications such as chlortetracycline and streptomycin are often used for treatment. Radiation therapy can be effective for early-stage lesions. Comprehensive treatment is generally administered based on the condition. If deformities develop during the cicatricial stage, surgical reconstruction may be performed. For pharyngeal or laryngeal lesions causing respiratory difficulty, a tracheotomy and surgery to remove the affected tissue are necessary. A patient is considered cured only after treatment when the scleroma bacillus tests negative, biopsies no longer show progressive scleromatous pathological changes, or the complement fixation reaction turns negative.

bubble_chart Differentiation

The catarrhal stage of this disease closely resembles atrophic rhinitis, atrophic pharyngitis, and atrophic laryngitis, but the mucous membrane is not severely atrophic and lacks a foul odor. On the atrophic mucous membrane, there are scattered granular granulations or hard infiltrations. It is also necessary to exclude syphilis, subcutaneous nodules, lupus vulgaris, malignant granuloma, tumors, keloids, and leprosy. Differentiating scleroma from malignant tumors is not difficult, although the two conditions may coexist. Some also believe that malignant tumors may develop on the basis of scleroma.