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Chapter 38

Scleroma (Rhinoscleroma)

Scleroma, or rhinoscleroma as it is known in some areas, is a chronic granulomatous infection of the nose and sometimes the upper respiratory tract caused by Klebsiella rhinoscleromatis. It follows an indolent course for many years, with occasional remissions and many relapses. In some patients, especially those with untreated or inadequately treated infections, death may result from airway obstruction, hemorrhage, or sepsis. The disease was initially described by von Hebra in 1874 in Austro-Hungary; he thought it was a form of skin cancer or "granulation sarcoma," although most investigators at that time thought it was a manifestation of syphilis. In 1882, von Frisch discovered the causative gram-negative bacilli which are found in the lesions. Although scleroma was first described in eastern Europe, it is today chiefly seen in the tropical regions of the world.

Lack of familiarity with scleroma in nonendemic areas, such as North America and western Europe, has undoubtedly resulted in a failure to recognize the entity, which may be confused with leprosy, syphilis, yaws, cutaneous leishmaniasis, South American blastomycosis, and malignant neoplasms.

Synonyms

Scleroma. Rhinoscleroma. Scrofulous lupus. Hebra nose. Sp: Rinoscleroma. Fr: Sclérome. Ger: Sklerom. Nasen-Rachenrauminduration

Definition

Scleroma is a chronic granulomatous disease in which painless inflammatory masses caused by Klebsiella rhinoscleromatis may develop in the nose and elsewhere in the respiratory tract.

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