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    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. Epub 2017 Jan 1.
    1 Department of Pathology, Hassan II University Hospital, Fès, Morocco.
    Rhinoscleroma is a chronic infectious disease that is endemic in certain poor areas of the world. The diagnosis often relies on pathology, the outcome is disappointing and then marked by recurrence. We present a retrospective series of six patients histologically diagnosed with rhinoscleroma. Read More

    Role of Histopathology in Differentiating Primary Atrophic Rhinitis from Atrophic Stage of Rhinoscleroma.
    Indian J Otolaryngol Head Neck Surg 2017 Mar 12;69(1):62-66. Epub 2017 Jan 12.
    Department of Community Medicine, Sri Devaraj Urs Medical College, Kolar, Karnataka India.
    Atrophic rhinitis (AR) is a chronic debilitating nasal mucosal disease of unknown aetiology and the definitive treatment is still elusive. This often leads to the patient suffering during the entire life span often depleting the individual psychologically. On the contrary, Rhinoscleroma (RS) for which the aetiology is clearly known and is completely curable has atrophic stage which is clinically indistinguishable from AR. Read More

    Granulomatous & histiocytic dermatitides.
    Semin Diagn Pathol 2017 May 14;34(3):301-311. Epub 2016 Dec 14.
    Section of Dermatopathology, Division of Surgical & Cytological Pathology, University of Virginia Medical Center, Charlottesville, VA, USA. Electronic address:
    Granulomas of the skin may be classified in several ways. They are either infectious or non-infectious in character, and they contain areas of necrobiosis or necrosis, or not. Responsible infectious agents may be mycobacterial, fungal, treponemal, or parasitic organisms, and each case of granulomatous dermatitis should be assessed histochemically for those microbes. Read More

    Rhinoscleroma with Pharyngolaryngeal Involvement Caused by Klebsiella ozaenae.
    Case Rep Infect Dis 2016 12;2016:6536275. Epub 2016 May 12.
    Department of Gastroenterology, University of Iowa Hospital and Clinics, Iowa City, IA 52242, USA.
    Rhinoscleroma is a chronic, slowly progressive granulomatous bacterial infection that is endemic to the tropical world, namely, Central America and Africa. It is occasionally seen in the United States of America (USA). It predominately affects the nasal mucosa but can also involve the rest of the upper respiratory tract. Read More

    A case of lobar pneumonia and sepsis with death caused by invasive Klebsiella rhinoscleromatis infection.
    J Infect Chemother 2016 Oct 14;22(10):707-11. Epub 2016 May 14.
    Kyushu General Internal Medicine Center, Haradoi Hospital, Fukuoka, Japan.
    Klebsiella pneumoniae often causes pneumonia and other infections in heavy drinkers and patients with diabetes. Pneumonia caused by Klebsiella rhinoscleromatis, a subspecies of K. pneumoniae, has not been previously reported. Read More

    Tracheal rhinoscleroma.
    Eur Ann Otorhinolaryngol Head Neck Dis 2016 Feb 4;133(1):51-3. Epub 2016 Jan 4.
    Département de chirurgie cervico-faciale, centre hospitalo universitaire, hôpital Gui-de-Chauliac, 80, avenue Augustin-Fliche, 34295 Montpellier cedex 5, France.
    Introduction: Tracheal rhinoscleroma is an infectious granulomatosis of the tracheobronchial tract caused by a Gram-negative bacillus. Exclusively tracheal involvement has been rarely reported in the literature. The purpose of this study was to report a case of subglottic stenosis secondary to rhinoscleroma. Read More

    Rhinoscleroma: a detailed histopathological diagnostic insight.
    Int J Clin Exp Pathol 2015 1;8(7):8438-45. Epub 2015 Jul 1.
    Department of Ear Nose and Throat, Faculty of Medicine, Sohag University Sohag, Egypt.
    Rhinoscleroma (RS) is a chronic specific disease of nose and upper respiratory passages caused by Klebsiella rhinoscleromatis bacilli. It is endemic in Egypt and in sporadic areas worldwide. Diagnosis of RS depends on identification of the pathognomonic Mickulicz cells (MCs) which is most prominent during granulomatous phase but spares or absent during catarrhal or sclerotic phases of the disease. Read More

    The involvement of upper airway in Wegener's granulomatosis - about four cases.
    Rom J Morphol Embryol 2015 ;56(2):613-8
    Department of ENT, "Victor Babes" University of Medicine and Pharmacy, Timisoara, Romania;
    The authors present four cases of Wegener's granulomatosis patients with multiorganic manifestation forms, but with a prevalent involvement in upper-airway. Granulomatosis diseases of the nose include bacterial infections (rhinoscleroma, tuberculosis, syphilis, lupus, and leprosy), fungal infections (rhinosporidiosis, aspergillosis, mucormycosis, candidosis, histoplasmosis, and blastomycosis) and diseases with unspecified etiology (Wegener's granulomatosis, mediofacial malignant granuloma, and sarcoidosis). We consider an interesting experience regarding Wegener's granulomatosis due to its rarity, being an autoimmune systemic disease, with continuous evolution and multiorganic involvement. Read More

    Topical mitomycin C as an adjunct to surgical debulking and medical treatment in rhinoscleroma.
    Am J Rhinol Allergy 2015 Jul-Aug;29(4):e95-9
    Otolaryngology, Head and Neck Department, Minia University, Minia, Egypt.
    Background: Rhinoscleroma is a chronic granuloma that is endemic in Egypt and is caused by gram-negative bacilli (Klebsiella rhinoscleromatis). The nasal mucosa is affected in almost all cases, which causes nasal obstruction, anosmia, and epistaxis. The disease usually passes through an atrophic stage (atrophic rhinitis) and a granulomatous stage before ending in the fibrotic stage, with possible bone destruction. Read More

    Airway scleromas and their extensions.
    ANZ J Surg 2016 Sep 26;86(9):670-4. Epub 2015 May 26.
    Department of Ear, Nose and Throat, Port Moresby General Hospital, Boroko, Papua New Guinea.
    Background: Scleroma is a rare, chronic, granulomatous infectious disease of the respiratory tract mucosa which begins in the nose and spreads to the respiratory tract and adjoining structures. We report on the extensions and the management of 134 cases of scleroma in the Highlands region of Papua New Guinea.

    Methods: The charts and treatment records of 134 scleroma cases were retrospectively reviewed from 1995 to 2013. Read More

    Rhinoscleroma of nose extruding into oral cavity.
    J Coll Physicians Surg Pak 2015 Apr;25 Suppl 1:S27-9
    Department of Oral Pathology and Microbiology, P.M.N.M. Dental College and Hospital, Bagalkot, Karnataka, India.
    Rhinoscleroma (RS) is a rare chronic granulomatous disease of the upper airways affecting nasal cavity, nasopharynx, and paranasal sinuses. Klebsiella rhinoscleromatis is the causative agent of this infection and Mikulicz cells are specific to this lesion. RS is commonly seen in poorer regions such as Central Africa, South America, Middle East, India and Indonesia. Read More

    Rhinoschleroma.
    Am J Trop Med Hyg 2015 Jan;92(1)
    Infectious Diseases Section, Internal Medicine Department, and Microsurgery and Oncology Plastic Surgery Department, University of Antioquia Medical School, Medellín, Colombia.
    A 39 year-old man came to our institution because of a five-year history of a progressive painful growing mass on his left nostril, which cause airway obstruction with ulceration. Because of a suspicion of malignancy, surgery (mass resection and subtotal nasal reconstruction) was performed. Histologic samples ruled out malignancy, and tissue cultures for fungus and mycobacteria were negative, but regular aerobic cultures were positive for Klebsiella rhinoscleromatis. Read More

    Granulomatous disease in the head and neck: developing a differential diagnosis.
    Radiographics 2014 Sep-Oct;34(5):1240-56
    From the Departments of Radiology (O.K.N., R.N., A.F., O.S.) and Otolaryngology-Head and Neck Surgery (O.S.), Boston Medical Center, Boston University School of Medicine, 820 Harrison Ave, Boston, MA 02118.
    Granulomatous diseases have a varied etiology that includes autoimmune, infectious, idiopathic, and hereditary causes. The unifying factor in these diseases is the formation of granulomas, which histologically are mononuclear inflammatory cells or macrophages surrounded by lymphocytes. Granulomatous diseases often have systemic manifestations that affect organs throughout the body. Read More

    The molecular genetics of inflammatory, autoimmune, and infectious diseases of the sinonasal tract: a review.
    Arch Pathol Lab Med 2014 Jun;138(6):745-53
    From the Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia.
    Context: The sinonasal tract is frequently affected by a variety of nonneoplastic inflammatory disease processes that are often multifactorial in their etiology but commonly have a molecular genetic component.

    Objective: To review the molecular genetics of a variety of nonneoplastic inflammatory diseases of the sinonasal tract.

    Data Sources: Inflammatory lesions of the sinonasal tract can be divided into 3 main categories: (1) chronic rhinosinusitis, (2) infectious diseases, and (3) autoimmune diseases/vasculitides. Read More

    Unexpected pathologies in patients referred for endoscopic DCR.
    Eur Arch Otorhinolaryngol 2014 Nov 30;271(11):3053-8. Epub 2014 Jan 30.
    Department of Ophthalmology, The Oculoplastic Institute, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel,
    The objective of this study is to describe a series of patients with different pathologies mimicking nasolacrimal duct obstruction (NLDO), diagnosed with the use of a computed tomography (CT) scan prior to a scheduled endoscopic dacryocystorhinostomy (DCR). This study is a retrospective report. We reviewed the medical records of 47 consecutive patients (57 sides) with long-standing epiphora between 2007 and 2012. Read More

    Rhinoscleroma in a 5-year-old Portuguese Child.
    Pediatr Infect Dis J 2014 Jul;33(7):774-5
    From the *Infectious Disease Unit, Department of Pediatrics H. Dona Estefânia, Centro Hospitalar Lisboa Central, Lisbon; and †Otorhinolaryngology Department, Hospital Distrital de Santarém, Santarém, Portugal.
    Rhinoscleroma is a chronic granulomatous infectious disease that is rare in Western Europe. We report the case of a 5-year-old Portuguese boy diagnosed with rhinoscleroma in the context of recurrent epistaxis. He had a 6-month course of antibiotic (amoxicillin plus clavulanate) therapy with full recovery. Read More

    Rhinoscleroma presenting as a nasal-palatal mass with airway obstruction.
    F1000Res 2013 9;2:124. Epub 2013 May 9.
    Department of Pathology, Washington Adventist Hospital, Takoma Park, Maryland, 20912, USA.
    We report a case of a 45-year-old male with severe rhinoscleroma. The patient presented to the emergency room with dyspnea from a long-standing nasal-palatal mass. A tracheostomy was required for airway control. Read More

    [Expression and significance of IgG4 in inflammatory disease of nasal cavity and paranasal sinuses].
    Zhonghua Bing Li Xue Za Zhi 2013 Jun;42(6):386-91
    Department of Pathology, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China.
    Objective: To study the prevalence of IgG4-positive plasma cells in inflammatory disease of nasal cavity and paranasal sinuses and its association with IgG4-related sclerosing disease (IgG4-SD).

    Methods: The expression of IgG4 and IgG in plasma cells of 103 cases diagnosed as inflammatory disease of nasal cavity and paranasal sinuses with dense lymphoplasmacytic infiltrate was studied by immunohistochemistry (EnVision) and quantitatively analyzed by medical image analysis system.

    Results: Immunohistochemical study showed marked infiltration by IgG4-positive plasma cells (>50 per high-power field) in 28 cases, moderate infiltration (30 to 50 per high-power field) in 23 cases, mild (10 to 29 per high-power field) in 30 cases and negative (<10 per high-power field) in 22 cases (P < 0. Read More

    Histopathological study of lesions of nose and paranasal sinuses.
    Indian J Otolaryngol Head Neck Surg 2012 Sep 10;64(3):275-9. Epub 2011 Sep 10.
    Department of Pathology, RCSM Government Medical College & CPR Hospital, Kolhapur, Maharashtra 416 002 India.
    (1) To study the incidence of benign and malignant lesions of nose and paranasal sinuses (PNS). (2) To study various lesions in reference to sex differences and symptomatology. (3) To compare the findings of the study with other authors. Read More

    Rhinoscleroma: a case series report and review of the literature.
    Eur Arch Otorhinolaryngol 2014 Jul 1;271(7):1851-6. Epub 2013 Aug 1.
    Otolaryngology Department, University Teaching Hospital (CHUK), National University of Rwanda, Kigali, Rwanda.
    Rhinoscleroma is a rare chronic granulomatous infectious disease and important differential diagnosis in developing countries and emigrants from these regions. In this survey we present seven cases from University Hospital Kigali, Rwanda, and one more from Germany. All cases are discussed on the background of the current literature. Read More

    [Giant rhinoscleroma].
    Rev Stomatol Chir Maxillofac Chir Orale 2013 Jun 26;114(3):184-6. Epub 2013 Mar 26.
    Service de chirurgie plastique reconstructrice et esthétique, chirurgie de la main et brûlologie, CHU de Treichville, Abidjan 01, Côte-d'Ivoire.
    Unlabelled: Rhinoscleroma is a chronic granulomatous respiratory tract disease. The initial lesion site is often intra-nasal. Giant tumor presentations are rare. Read More

    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
    Institut Pasteur, Génotypage des Pathogènes et Santé Publique, Paris Cedex, France.
    Rhinoscleroma is a human specific chronic disease characterized by the formation of granuloma in the airways, caused by the bacterium Klebsiella pneumoniae subspecies rhinoscleromatis, a species very closely related to K. pneumoniae subspecies pneumoniae. It is characterized by the appearance of specific foamy macrophages called Mikulicz cells. Read More

    Rhinoscleroma in an immigrant from Egypt: a case report.
    J Travel Med 2012 Dec 11;19(6):387-90. Epub 2012 Oct 11.
    Department of Pathological Anatomy, Niguarda Cà Granda Hospital, Milano, Italy.
    Rhinoscleroma is a chronic indolent granulomatous infection of the nose and the upper respiratory tract caused by Klebsiella rhinoscleromatis; this condition is endemic to many regions of the world including North Africa. We present a case of rhinoscleroma in a 51-year-old Egyptian immigrant with 1-month history of epistaxis. We would postulate that with increased travel from areas where rhinoscleroma is endemic to other non-endemic areas, diagnosis of this condition will become more common. Read More

    [One case report of Rosai-Dorfman disease in nasal cavity and nasal sinuses].
    Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2012 Nov;26(21):1007-8
    Rosai-Dorfman disease in nasal cavity and nasal sinuses is known as an idiopathic proliferative disease of histiocytes with a distinct morphologic feature and is very rare. It is easy to relapse and be misdiagnosed. In on case, the infiltrating histiocytes had large round or oval nuclei. Read More

    [Rhinoscleroma: two cases in Bobo-Dioulasso in Burkina Faso].
    Med Sante Trop 2012 Oct-Dec;22(4):409-11
    CHU Souro-Sanou, service ORL, Bobo-Dioulasso, Burkina Faso.
    Introduction: Rhinoscleroma is a chronic granulomatous infection of the respiratory tract due to Klebsiella rhinoscleromatis (K. rhinoscleromatis).

    Patients And Method: We observed two cases of rhinoscleroma in the Souro-Sanou university hospital of Bobo-Dioulasso, Burkina Faso, over a 9-year period (2009 to 2010). Read More

    Surgical management of scleromatous laryngotracheal stenosis.
    Auris Nasus Larynx 2013 Aug 29;40(4):388-93. Epub 2012 Dec 29.
    Department of Oto-Rhino-Laryngology, Military Medical Academy, Cairo, Egypt.
    Objective: Scleroma is a chronic specific granulomatous disease of the upper respiratory tract caused by Klebsiella Rhinoscleromatis. It usually affects the subglottic region and upper trachea resulting in various degrees of stenosis. Patients with laryngotracheal stenosis may present with stridor, shortness of breath or exercise intolerance and may be tracheostomy dependent. Read More

    Role of MR imaging in laryngoscleroma.
    Eur Arch Otorhinolaryngol 2013 Mar 4;270(3):985-8. Epub 2012 Nov 4.
    Department of Diagnostic Radiology, Mansoura Faculty of Medicine, Mansoura, Egypt.
    To evaluate the role of MR imaging in patients with laryngoscleroma. We retrospectively reviewed the MR imaging of 14 patients (11 female, 3 male with mean age of 31 years) with pathologically proven laryngoscleroma. They presented with dysphonia (n = 12), stridor (n = 8) and airway obstruction (n = 4). Read More

    A rare case of primary otoscleroma of the middle ear.
    J Laryngol Otol 2012 Dec 4;126(12):1276-7. Epub 2012 Oct 4.
    Department of ENT, Al-Ameen Medical College, Bijapur, Karnataka, India.
    Objective: We report an extremely rare case of primary otoscleroma.

    Method: We present a case report and a review of the world literature concerning otoscleroma.

    Results: An adult woman presented with chronic suppurative otitis media with tubotympanic disease and conductive hearing loss. Read More

    Imaging of scleroma in the head and neck.
    Br J Radiol 2012 Dec 16;85(1020):1551-5. Epub 2012 Aug 16.
    Department of Diagnostic Radiology, Mansoura Faculty of Medicine, Egypt.
    We review the appearance of scleroma in the head and neck on imaging. Scleroma is a chronic granulomatous disease that primarily affects the nasal cavity, but the pharynx and larynx may also be involved. On imaging, nasal scleroma appears as bilateral or unilateral expanded homogeneous nasal masses that may exhibit hyperintense signal on T(1) weighted images. Read More

    Coexistence of rhinoscleroma with Rosai-Dorfman disease: is rhinoscleroma a cause of this disease?
    J Laryngol Otol 2012 Jun;126(6):630-2
    Department of ENT, Co-Operative Medical College, Kochi, Kerala, India.
    Introduction: Rhinoscleroma and Rosai-Dorfman disease have been reported to coexist in the same patient at different sites. Rosai-Dorfman disease may have an aetiological relationship with rhinoscleroma, although this has not yet been proved.

    Case Report: A case of a 42-year-old woman with recurrent nasal masses is presented, with histopathological proof of both conditions coexisting in the same nasal lesion. Read More

    [Extranodal Rosai-Dorfman disease of upper respiratory tract: a clinicopathologic study].
    Zhonghua Bing Li Xue Za Zhi 2012 Jan;41(1):11-5
    Department of Pathology, Eye and Ear Nose Throat Hospital of Fudan University, Shanghai 200031, China.
    Objective: To study the clinicopathologic features and differential diagnosis of extranodal Rosai-Dorfman disease (RDD) of the upper respiratory tract.

    Methods: The clinical, pathologic and immunohistochemical features of 10 cases of RDD were evaluated.

    Results: Among the 10 cases studied, there were 3 males and 7 females. Read More

    Rhinoscleroma: a case series.
    Singapore Med J 2012 Feb;53(2):e24-7
    National Skin Centre, 1 Mandalay Road, Singapore.
    Rhinoscleroma is a chronic, slowly progressive, inflammatory disease of the upper respiratory tract. It is associated with Klebsiella rhinoscleromatis infection. We present the clinical and pathological features of four patients diagnosed with rhinoscleroma at the National Skin Centre, Singapore between 1997 and 2010. Read More

    A mild form of SLC29A3 disorder: a frameshift deletion leads to the paradoxical translation of an otherwise noncoding mRNA splice variant.
    PLoS One 2012 4;7(1):e29708. Epub 2012 Jan 4.
    St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, New York, United States of America.
    We investigated two siblings with granulomatous histiocytosis prominent in the nasal area, mimicking rhinoscleroma and Rosai-Dorfman syndrome. Genome-wide linkage analysis and whole-exome sequencing identified a homozygous frameshift deletion in SLC29A3, which encodes human equilibrative nucleoside transporter-3 (hENT3). Germline mutations in SLC29A3 have been reported in rare patients with a wide range of overlapping clinical features and inherited disorders including H syndrome, pigmented hypertrichosis with insulin-dependent diabetes, and Faisalabad histiocytosis. Read More

    Cytohistological features of rhinoscleroma.
    Indian J Pathol Microbiol 2011 Oct-Dec;54(4):806-8
    Department of Pathology and Lab Medicine, Deen Dayal Upadhya Hospital, Hari Nagar, New Delhi, India.
    Rhinoscleroma, also known as Mikulicz disease, is a chronic progressive disease caused by Klebsiella rhinoscleromatis. The disease is uncommon in Delhi. There are no cytological references of this disease. Read More

    Jan Mikulicz-Radecki (1850-1905): his impact on modern medicine.
    Clin Dermatol 2012 Jan-Feb;30(1):129-36
    Department of Ophthalmology, Poznań City Hospital, ul. Szwajcarska 3, 61-285 Poznań, Poland.
    Abstract Jan Mikulicz-Radecki (1850-1905) was the cofounder of modern surgery, aseptic techniques,and the inventor of gastroscopy, but his professional accomplishments go far beyond the field of surgical treatment. Various medical achievements are named in his honor, including the name of the cells that he discovered in rhinoscleroma, the name of Mikulicz disease, and the name of an ointment that he developed and continues to be used in the treatment of wounds and ulcers in surgery and dermatology. Noteworthy are also his interdisciplinary approach towards diagnosis and treatment, and his cordial attitude towards his patients. Read More

    PCR-based identification of Klebsiella pneumoniae subsp. rhinoscleromatis, the agent of rhinoscleroma.
    PLoS Negl Trop Dis 2011 May 24;5(5):e1052. Epub 2011 May 24.
    Institut Pasteur, Genotyping of Pathogens and Public Health, Paris, France.
    Rhinoscleroma is a chronic granulomatous infection of the upper airways caused by the bacterium Klebsiella pneumoniae subsp. rhinoscleromatis. The disease is endemic in tropical and subtropical areas, but its diagnosis remains difficult. Read More

    B lymphocyte subsets in patients with rhinoscleroma.
    Otolaryngol Head Neck Surg 2011 May;144(5):809-14
    Department of Otorhinolaryngology, Audiology, and Phoniatrics Giorgio Ferreri, Sapienza University of Rome, Rome, Italy.
    Objective: To identify the presence of B lymphocytes in the rhinoscleroma granulomas as a possible precursor of plasma cells, whose presence has always been described but whose role is still unclear.

    Study Design: Case series with chart review.

    Setting: Sapienza University of Rome. Read More

    Rhinoscleroma: a retrospective study of pathologic and clinical features.
    J Otolaryngol Head Neck Surg 2011 Apr;40(2):167-74
    Department of Otolaryngology-Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Key Laboratory of Otolaryngology Head and Neck Surgery, Beijing, China.
    Objective: Rhinoscleroma, a chronic granulomatous bacterial disease of the nasal mucosa that often extends through the lower respiratory tract, is caused by infection with the gram-negative bacillus Klebsiella rhinoscleromatis (KR). We report the clinicopathology and histology associated with KR infection-induced rhinoscleroma in patients admitted to the Beijing Tongren Hospital over a 30-year period.

    Methods: The clinical and pathologic features of 40 cases of upper aerodigestive tract infections were retrospectively studied. Read More

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