A Myriad of Scleroma Presentations: The Usual and Unusual
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ORIGINAL PAPER
A Myriad of Scleroma Presentations: The Usual and Unusual Samy Elwany1 · Hesham Abdel Fattah1 · Zeyad Mandour1 · Ahmed Soliman Ismail1 · Mostafa Abdelnabi1 Received: 17 May 2019 / Accepted: 6 September 2019 © Springer Science+Business Media, LLC, part of Springer Nature 2019
Abstract To prospectively study a series of scleroma patients with a focus on socioeconomic status, clinical presentation, pathology, microbiology, and treatment. A total of 52 patients diagnosed with scleroma were included in the study. Demographic data of the patients were collected and clinical, radiographic, and histopathologic findings were analyzed. Biopsies from atypical patients were sent for electron microscopic examination. Patients were treated both medically and surgically with clinical follow-up to determine outcomes and the incidence of recurrence. Fifty-two patients were included in the study. There were 23 males (44.2%) and 29 females (55.8%). The highest incidence of scleroma was identified in individuals from poor, rural areas in the third to fourth decades of life. The nose was affected in all cases. Other affected sites included the nasopharynx, soft and hard palate, larynx and trachea, lacrimal passages, skin, and gingiva. Some patients had atypical presentations. Complete follow-up of all patients was hindered by non-compliance with clinic visits and the long duration of medical treatment. Recurrence or relapse of the granulomatous lesions after initial improvement occurred in 11 patients (21.2%) within 1 to 3 years. Scleroma is a stubborn, chronic, granulomatous bacterial disease. The diverse presentations may mimic neoplasms and other granulomatous conditions. We present cases with unusual presentations and demonstrate the transmission electron microscopic features of these lesions. Furthermore, we confirm the importance of achieving full eradication of the etiologic bacilli to prevent recurrent disease. Keywords Rhinoscleroma · Granuloma · Nose · Klebsiella · Tracheostomy
Introduction Scleroma is a chronic granulomatous disease of the upper respiratory tract caused by a gram-negative bacillus, Klebsiella rhinoscleromatis (Von Frisch bacillus) [1, 2]. In classic form, the disease affects the nose in nearly all cases hence the former name of rhinoscleroma. The disease is endemic in Egypt, Central and South America, South-East Asia, and areas of Eastern Europe [3, 4]. Despite affecting populations around the globe, epidemiologic data on the incidence and prevalence of scleroma in different regions of the world are lacking [2]. Scleroma frequently involves sites other than the nose. These include the pharynx, larynx, trachea, and bronchi [5, 6]. Atypical presentations of scleroma are occasionally reported in the literature. These include involvement of * Samy Elwany [email protected] 1
Department of Otolaryngology, Alexandria University, 4 Kafr Abdou Street #605, Alexandria 21529, Egypt
the sinuses, lacrimal apparatus, cervical lymph nodes, and cranial cavity as well as the development of
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