Clinical, radiological and pathological study of 88 cases of typical and complicated scleroma

Abstract

Background: Scleroma is a chronic specific granulomatous disease. It is considered endemic in Central America, Egypt, tropical Africa, India, and Indonesia. The most common complaint is nasal obstruction, other symptoms include; rhinorrhea, epistaxis, dysphagia, stridor, and dysphonia. Aim: So our objective was to focus on the various clinical and radiological manifestations of scleroma in an endemic area, with some atypical symptoms and signs, and to alert physicians to suspect scleroma or its complications in any case of unexplained nasal lesion. Material and methodology: Our study included 88 cases who were admitted in our otorhinolaryngology department. They presented with various manifestations of Rhino-pharyngo-laryngo scleroma, out of these patients 16 cases showed atypical presentations. Our diagnosis was based on clinical and radiological examination, aided by bacteriological and pathological examination. Results: Patients with typical rhinoscleroma (72 patients) presented with various nasal and laryngo-tracheal symptoms. 16 patients (18%) presented complaining of various nasal symptoms and signs, which were not suspected to be rhinoscleroma, so our workup was directed to diagnose those patients, and their final diagnosis was reached to be rhinoscleroma, we sub classified those patients according to their presentation. Conclusion: Rhinoscleroma can present clinically with atypical features, so in order to avoid misdiagnosis, it is important to keep in mind these atypical manifestations.Please cite this paper as: Fawaz S, Tiba M, Salman M, and Othman H. Clinical, radiological and pathological study of 88 cases of typical and complicated scleroma. Clin Respir J 2011; 5: 112-121.

Respiratory scleroma: A clinicopathologic study of 51 cases from Guatemala

“…Two large series from Egypt also show similarities with our data, especially in symptoms and sites affected (Fawaz et al, ; Gaafar et al, ). Interestingly, Fawaz et al () present a different set of “atypical” RS cases, with intracranial and intraorbital involvement. Atypical symptoms included forehead and cheek swelling, which were not found in our patients.…”

“…These cells appear after the non‐specific inflammatory reaction present in the atrophic stage, when bacteria are released into tissues after incomplete digestion and cell death (Fevre et al, ). In the fibrotic stage, the inflammatory infiltrate is replaced by extensive areas of fibrosis, when MC identification may become challenging (Fawaz, Tiba, Salman, & Othman, ).…”

“…Respiratory scleroma is endemic in poor regions of Central America, Egypt, tropical Africa, and Southeast Asia (Fawaz et al, ). However, national or international epidemiological data are scarce.…”

“…However, national or international epidemiological data are scarce. While hundreds of cases are reported in developing countries (Fawaz et al, ; Gaafar, Gaafar, & Nour, ; Quevedo, ), only a small number of patients are diagnosed with RS in developed ones (Andraca, Edson, & Kern, ; de Pontual et al, ). In this context, we aimed to perform a clinicopathologic study of RS in Guatemala.…”

Central airway pathology: clinic features, CT findings with pathologic and virtual endoscopy correlation

“…The disease tends to progress slowly in periods of remission and relapse through three overlapping stages: the rhinitic stage, granulomatous stage with plasma cells and Mikulicz cells (histiocytes containing K. rhinoscleromatis bacilli ) and fibrotic stage, characterised by scar tissue [28, 50]. A positive K. rhinoscleromatis bacilli culture is diagnostic for the disease but occurs in less than 60% of cases [51].…”