Rhinosclerosis A48.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 15.06.2022

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Synonym(s)

nasal scleroma; Rhinoscleroma

History
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v.Hebra, 1870

Definition
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Rare, chronic, bacterial (Klebsiella pneumoniae rhinoscleromatis - see Klebsiella below) infectious disease of the nose, oral mucosa and upper respiratory tract (Umphress B et al.).

Pathogen
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Klebsiella pneumoniae subsp. rhinoscleromatis an immobile, encapsulated, Gram-negative rod. Transmission from person to person by droplet infection.

Occurrence/Epidemiology
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China, India, Central Africa, Central and South America.

Manifestation
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Mainly occurring between the ages of 20 and 35.

Clinical features
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Rhinitic pre-stage with foetal nasal secretion, crusts, dry nasal and pharyngeal mucosa.

Later crusts and inflammatory hard infiltrates develop in the nasal mucosa, upper lip, pharynx and larynx.

Development of hard, reddish, painless, vegetative granulations with fusion to unshaped, nodular formations (Hebra- or tapir nose). The infectious granulomas can destroy adjacent bony structures. Undisturbed general condition.

Histology
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Chronic inflammation in the middle corium. Dense accumulation of plasma cells, Russel corpuscles and Mikulicz cells.

Diagnosis
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Cultural detection of bacteria, if necessary animal experiments (mice).

Differential diagnosis
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External therapy
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Sprayscontaining glucocorticoids such as Nasonex, budesonide nasal spray(e.g. Pulmicort Topinasal) 1 spray in each nostril in the morning and evening. Moist compresses with antiseptic additives such as quinolinol (e.g. Chinosol 1:1000) or potassium permanganate (light pink) on externally accessible affected skin areas.

Internal therapy
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Antibiosis e.g. with Ciprofloxacin 250-500mg p.o. over 6-8 weeks.

Alternatively Tetracycline (e.g. Tetracycline 500 Wolff 2-4 times/day 1 Kps.) or Minocycline (e.g. Mino-Wolff) 2 times/day 100 mg p.o. over 6 months.

Alternatively: Cotrimoxazol p.o. (e.g. Eusaprim® forte 3 times/day 1 tbl.).

Alternative: Clofazimine (e.g. Lamprene) initial 50-100 mg/day p.o., maintenance therapy with 50 mg/day (under maintenance dose of 50 mg practically no NW).

Operative therapie
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Removal of all sclerotic infiltrates or nodes is a prerequisite for subsequent drug therapy. Possibly also therapy with the CO2 laser after completion of the antibiotic therapy, if necessary reconstructive measures.

Literature
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  1. Hebra F v (1870) About a strange new formation on the nose - rhinoscleroma. Vienna Med Weekly 20: 1-4
  2. Kasper HU et al (2004) Rhinoscleroma associated with Rosai-Dorfman reaction of regional lymph nodes. Catholic Int 54: 101-104
  3. Umphress B et al (2018) Rhinoscleroma. Arch Catholic Lab Med 142:1533-1536.

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 15.06.2022