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Clinical Characteristics

Scleroma produces spontaneous, painless, chronic inflammatory nodules, most commonly in the nose, but in some patients anywhere along the respiratory passages from the nostrils to the pulmonary hila. These nodules grossly deform the nose and narrow the respiratory passages. The disease has an insidious onset, developing slowly with little or no effect on the general health of the individual. It may sometimes enter a quiescent phase, only to subsequently become reactivated.

Scleroma usually progresses through three distinct stages: (1) the exudative, catarrhal, or rhinitis stage; (2) the proliferative, granulomatous, or nodular stage; and (3) the fibrotic or cicatricial stage.

The initial stage of rhinitis is characterized by watery nasal discharge, fronto-ethmoidal headaches and difficulty in breathing with nasal obstruction and epistaxis. The nasal mucosa may be hyperemic, edematous or hypertrophic, or there may be a thin, dry mucous membrane covered with a yellow crust. Tiny, discrete, bluish-red edematous nodules may be noted. Sore throat and hoarseness may be present if the nasopharynx and larynx are involved.

This stage may persist for months or years and gradually gives way to the second, florid, nodular stage, which is characterized by the disappearance of coryza, by progressive infiltration, and by coalescence of the nodules to form large granular masses. These waxy, hard nodules arise from the floor of the nose and the nasal septum, which enlarges. Over a period of months or years, there is slow spread of the nodules to involve both the medial and lateral walls of the nose, leading to nasal obstruction. As the granulomatous process progresses, these hard, nonpliable masses of granulation tissue cause a grotesque deformity of the walls of the nose and may spread backwards to involve the palate with loss of the palatal reflex. Continued growth of the nasal lesions causes expansion of the anterior nares and deformity of the upper lip to produce the characteristic nasal configuration called the "Hebra nose" (after von Hebra, who first described the disease) (Fig. 38.3).

Fig. 38.3 Typical "Hebra nose" deformity seen in some patients in the granulomatous or nodular stage of rhinoscleroma. AFIP 66153. (Courtesy of Dr. H. K. Giffen; from C.H. Binford and D.H. Connor (eds): Pathology of Tropical and Extraordinary Diseases. Armed Forces Institute of Pathology, Washington DC, 1976).

When the granulomatous process grows upwards, nasal respiration is affected, but when it is directed inferiorly, the nose may be completely obstructed. Anosmia results from forward extension and the firm masses may sprout out of the nostrils to involve the nasal tip, alae, upper lip, and gums. In advanced stages of the disease, these nasal lesions may bleed easily and slightly ulcerate and become secondarily infected, leading to extensive, often hideous destruction of the entire nose and upper lip. The tissues become indurated and have a cartilaginous consistency. The patient thus affected may eventually succumb from asphyxiation, infection such as aspiration bronchopneumonia, hemorrhage, or debilitation.

In the third or cicatricial stage of the disease, the masses gradually become avascular. Adhesions of the soft structures and further cicatricial contraction of the scleromatous tissue may lead to stenosis and virtually occlude the nasopharynx, larynx and air passages, causing death from severe airways obstruction.

The initial symptoms of scleroma, consisting primarily of rhinitis, may resemble the common cold and, in the early stages, the granularity of the nasal mucosa may resemble an atrophic rhinitis. The later symptoms in the infiltrative or nodular stage depend on the area affected and the extent and duration of involvement. Although scleroma usually begins in the nose and paranasal sinuses with gradual extension downwards to involve other areas of the respiratory tract, lesions of the nasal septum, upper lip, cheek, gums, lacrimal ducts, eustachian tubes, nasopharynx, hard and soft palate, tonsils, pharynx, larynx, trachea, and bronchi have all been reported.

Airway obstruction, dysphonia or aphonia, and anosmia are the dysfunctional manifestations of scleroma in most patients. Interference with chewing and swallowing, speech defects, respiratory tract infections, tinnitus, deafness, and headache have all been described. The vascular granulomatous phase may cause laryngeal and tracheal hemorrhage. Anesthesia of the soft palate and enlargement of the uvula may suggest scleroma clinically at times (although in other patients there is perforation of the nasal septum and total destruction of the uvula). The orbit is occasionally involved, leading to exophthalmos and blindness, but intracranial extension is rare. Destruction of bone and soft tissue from compression may suggest a malignant neoplasm both clinically and radiologically.

Late in the disease, the clinical symptoms may relate also to the cicatricial changes that persist after the disease has burned out or responded to antibiotic therapy. Spontaneous regressions have rarely been seen, chiefly in patients whose disease is localized in the nasal septum and who show residual nasal atrophy. Once established, however, scleroma is a slowly progressive disease which may persist for several decades. If untreated or inadequately treated, the patient may eventually die as a result of airway obstruction, hemorrhage or, rarely, intracranial extension. In most patients, although the granulomatous process extends along the respiratory passages, there is little change in the surrounding tissues other than occasional enlargement of neighboring lymph glands, and the patient's general health is largely unaffected. There have been recent reports, however, of one person with systemic K. rhinoscleromatis infection and two HIV-infected patients with oropharyngeal rhinoscleroma.

Many modes of therapy have been tried in the scleroma patient, consisting primarily of antibiotics, radiation therapy, and surgical procedures for relief of obstruction or plastic repair of the airway. Radiation therapy has been used successfully, but antibiotic treatment with streptomycin, tetracycline, and chloramphenicol has been useful in arresting the disease in many patients.

In sum, the clinical findings of chronic, indurated, nodular masses, stenosis, and deformity, especially of the nose, in the absence of significant ulceration, systemic involvement, or pain should alert the clinician to the diagnosis of scleroma.

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