History

Rhinoscleroma is rare chronic granulomatous infection that should be considered in patients from countries in which the disease is endemic if they have nasal polyps that significantly adhere to the nasal septum with relative sparing of the sinuses. Most often, the presentation is nonspecific. Because of its mundane clinical presentation resembling chronic rhinitis, it often goes unrecognized.^[17]Chronic nasal infection caused by K rhinoscleromatis is often a misdiagnosed infectious disease.^[18]Chronic rhinitis, even in developed countries, should prompt its consideration, especially because specific diagnostic tools and effective treatments are available. Recurrent epistaxis may be evident.^[14]The pharynx, larynx, hard and soft palates, maxillary sinuses, and upper lip may be involved.^{[19, 20]}

Note the following possible history findings:

Nasal obstruction (most common complaint)
Rhinorrhea
Epistaxis
Dysphagia
Nasal deformity
Anesthesia of the soft palate
Difficulty breathing that progresses to stridor
Dysphonia
Anosmia

Physical Examination

The initial nodule is often intra-nasal and small in size.^[1]Rarely, if neglected, it can grow into an exophytic giant tumor, which may obstruct the entire respiratory tract.^[21] Rarely, rhinoscleroma of the nasal cavity may extrude into the oral cavity.^[22]Rhinoscleroma may also extend into the orbit, to the base of the skull, and into the brain.^[23]It may enter the brain through the cribriform plate.

Complications

Rhinoscleroma is a rare cause of upper airway obstruction. Isolated tracheal obstruction.^{[24, 25]}Subglottal stenosis may be a late sequela of rhinoscleroma.^[26]Scleroma is known to cause slowly progressive asphyxia.

Differential Diagnoses

de Pontual L, Ovetchkine P, Rodriguez D, et al. Rhinoscleroma: a French national retrospective study of epidemiological and clinical features. Clin Infect Dis. 2008 Dec 1. 47(11):1396-402. [QxMD MEDLINE Link].
Efared B, Hammas N, Gabrielle AE, Ben Mansour N, El Fatemi H, Chbani L. Rhinoscleroma: a chronic infectious disease of poor areas with characteristic histological features - report of a series of six cases. Trop Doct. 2017 Jan 1. 49475517704612. [QxMD MEDLINE Link].
Corelli B, Almeida AS, Sonego F, Castiglia V, Fevre C, Brisse S, et al. Rhinoscleroma pathogenesis: The type K3 capsule of Klebsiella rhinoscleromatis is a virulence factor not involved in Mikulicz cells formation. PLoS Negl Trop Dis. 2018 Jan. 12 (1):e0006201. [QxMD MEDLINE Link].
Sadek HS, El-Marssafy LH, Hussein FF. First report of rare persistence granulomatous extra respiratory Rhinoscleroma of oral pathology. Microb Pathog. 2022 Mar. 164:105451. [QxMD MEDLINE Link].
Podder I, Gharami RC. Penile Rhinoscleroma Masquerading as Penile Malignancy: A Case of Mistaken Identity. Indian J Dermatol. 2021 Jul-Aug. 66 (4):409-411. [QxMD MEDLINE Link]. [Full Text].
De Champs C, Vellin JF, Diancourt L, et al. Laryngeal scleroma associated with Klebsiella pneumoniae subsp. ozaenae. J Clin Microbiol. 2005 Nov. 43(11):5811-3. [QxMD MEDLINE Link].
Sanchez-Marin LA, Bross-Soriano D, Arrieta J, et al. Association of HLA-DQA1*03011-DQB1*0301 haplotype with the development of respiratory scleroma. Otolaryngol Head Neck Surg. 2007 Mar. 136(3):481-3. [QxMD MEDLINE Link].
Fevre C, Almeida AS, Taront S, Pedron T, Huerre M, Prevost MC, et al. A novel murine model of rhinoscleroma identifies Mikulicz cells, the disease signature, as IL-10 dependent derivatives of inflammatory monocytes. EMBO Mol Med. 2013 Apr. 5(4):516-30. [QxMD MEDLINE Link]. [Full Text].
Efared B, Hammas N, Gabrielle AE, Ben Mansour N, El Fatemi H, Chbani L. Rhinoscleroma: a chronic infectious disease of poor areas with characteristic histological features - report of a series of six cases. Trop Doct. 2018 Jan. 48 (1):33-35. [QxMD MEDLINE Link].
Abalkhail A, Satti MB, Uthman MA, Al Hilli F, Darwish A, Satir A. Rhinoscleroma: a clinicopathological study from the Gulf region. Singapore Med J. 2007 Feb. 48(2):148-51. [QxMD MEDLINE Link].
Azevedo-Bernarda R. Rhinoscleroma. Cent Afr J Med. 1971 Nov. 17(11):225-8. [QxMD MEDLINE Link].
Chan TV, Spiegel JH. Klebsiella rhinoscleromatis of the membranous nasal septum. J Laryngol Otol. 2007 Oct. 121(10):998-1002. [QxMD MEDLINE Link].
Navazo Eguia AI, Garcia Vicario F. [Rhinoscleroma]. Acta Otorrinolaringol Esp. 2010 Mar-Apr. 61(2):160-2. [QxMD MEDLINE Link].
Simão I, Gaspar I, Faustino R, Brito MJ. Rhinoscleroma in a 5-year-old Portuguese Child. Pediatr Infect Dis J. 2014 Jul. 33(7):774-5. [QxMD MEDLINE Link].
Gamea AM, El Tatawi FAY, Gamea MA. Changes in the pattern of rhinoscleroma in Egypt: updated experience. J Laryngol Otol. 2019 Sep 19. 1-5. [QxMD MEDLINE Link].
Gaafar HA, Gaafar AH, Nour YA. Rhinoscleroma: An updated experience through the last 10 years. Acta Otolaryngol. 2011 Apr. 131(4):440-6. [QxMD MEDLINE Link].
Salima K, Samia S, Mounir T, Fethi J, et al. [Rhinoscleroma: a report of 2 cases]. Tunis Med. 2005 Sep. 83(9):568-71. [QxMD MEDLINE Link].
Botelho-Nevers E, Gouriet F, Lepidi H, et al. Chronic nasal infection caused by Klebsiella rhinoscleromatis or Klebsiella ozaenae: two forgotten infectious diseases. Int J Infect Dis. 2007 Sep. 11(5):423-9. [QxMD MEDLINE Link].
Mariz BALA, Sánchez-Romero C, Romañach MJ, de Almeida OP, Carlos R. Respiratory scleroma: A clinicopathologic study of 51 cases from Guatemala. Oral Dis. 2020 Apr. 26 (3):670-676. [QxMD MEDLINE Link].
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Nayak P, R C P, K V S, Desai D, Pandit S, Ingaleshwar PS. Rhinoscleroma of nose extruding into oral cavity. J Coll Physicians Surg Pak. 2015 Apr. 25 (11):S27-9. [QxMD MEDLINE Link].
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Al Jahdali H, Bamefleh H, Memish Z, Al-Zuwayed M, Al Othman A. Upper airway obstruction due to rhinoscleroma: case report. J Chemother. 2001 Apr. 13 Suppl 1:69-72. [QxMD MEDLINE Link].
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Nagalotimath US, Naveen K, Puranik RB, Manjunath D, Venkatesha M. Role of Histopathology in Differentiating Primary Atrophic Rhinitis from Atrophic Stage of Rhinoscleroma. Indian J Otolaryngol Head Neck Surg. 2017 Mar. 69 (1):62-66. [QxMD MEDLINE Link].
Ma L, Liu GJ, Zhang TQ. Extranodal natural killer/T cell lymphoma with involvement of the bilateral submandibular lymph node mimicking a rhinoscleroma: a diagnostic challenge. J Stomatol Oral Maxillofac Surg. 2021 Feb. 122 (1):107-111. [QxMD MEDLINE Link].
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Media Gallery

Very high magnification micrograph of rhinoscleroma. Hematoxylin and eosin stain. Silver staining demonstrates the presence of microorganisms consistent with Klebsiella rhinoscleromatis. Courtesy of Nephron (own work), via Wikimedia Commons.

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Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Coauthor(s)

Egle Goriniene, MD Staff Physician, Department of Infectious Diseases, New Jersey Medical School

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) <br/>Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Jacek C Szepietowski, MD, PhD Professor, Vice-Head, Department of Dermatology, Venereology and Allergology, Wroclaw Medical University; Director of the Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Poland

Disclosure: Received consulting fee from Orfagen for consulting; Received consulting fee from Maruho for consulting; Received consulting fee from Astellas for consulting; Received consulting fee from Abbott for consulting; Received consulting fee from Leo Pharma for consulting; Received consulting fee from Biogenoma for consulting; Received honoraria from Janssen for speaking and teaching; Received honoraria from Medac for speaking and teaching; Received consulting fee from Dignity Sciences for consulting; .

Rhinoscleroma Clinical Presentation

History

Physical Examination

Complications

References

Tables

Contributor Information and Disclosures

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