Granulomatous Diseases of Nose

By : Ritu Rajan

The granulomatous conditions affecting the nose and sinuses can be classified as :

InfectiveBacterial

Rhinoscleroma Syphilis TuberculosisLupusLeprosy

Fungal Rhinosporidiosis AspergillosisMucormycosisHistoplasmosisCandidiasisBlastomycosis

InflammatoryWegener’s granulomatosis,SarcoidosisChurg-Strauss syndromeCholesterol GranulomaEosinophilic Granuloma

NeoplasticT-cell Lymphoma

(Non-healing midline granuloma)

• It is a chronic granulomatous disease caused by Gram-negative bacillus called Klebsiella Rhinoscleromatis or Frisch bacillus.

• It is also known as Mikulicz disease or Hebra Nose.

Rhinoscleroma

Etiological Factors –• It is more common in second and third decades of life, though any age can be affected.• The disease is endemic in several parts of the world (like India, Africa and Middle East).• There is a slight female preponderance throughout the world.• In India, it equally affects men and women.• In India, it is seen more often in the northern than in the southern parts.

Pathology -• Mode of infection is unknown.• The disease starts in the nose and extends to nasopharynx, oropharynx, larynx (mostly

subglottic region), trachea and bronchi.

Clinical Features-The four stages of this disease are :

1.Catarrhal : Foul smelling purulent nasal discharge for weeks to months.

2. Atrophic stage : The features resemble that of atrophic rhinitis, including crust formation and a foul smelling discharge (carpenter's glue).

3. Granulomatous stage : • Multiple granulomatous nodules, which enlarge

and coalesce, are seen in nasal mucosa.• Subdermal infiltration of lower part of external

nose and upper lip gives “woody” feel. • These are painless and non-ulcerative nodules.• They can be found in pharynx, larynx, trachea

and bronchi also.• They are initially bluish red and rubbery and

later become pale and indurated.• These nodules never break down but ultimately

fibrose and progressively decrease in size. • Regional lymph node involvement is extremely

infrequent.

4. Cicatricial stage :

Fibrosis leads to stenosis of nares, distortion of upper lip and adhesions in the nose, nasopharynx, oropharynx and larynx . The subglottic stenosis manifests as respiratory distress.

Diagnosis –Diagnosis in the atrophic stage is extremely difficult and the disease is usually recognized only in the granulomatous or cicatrizing stage.

1. Biopsy : Submucosa is infiltration with plasma cells, lymphocytes, eosinophils, Mikulicz cells and Russell bodies.

2. Cultures of infected tissue: The causative organisms are cultured and are diagnostic.

1) Mikulicz cells – These are large foam cells with a central nucleus and vacuolated cytoplasm containing causative bacilli.

2) Russell bodies - These are homogenous eosinophilic inclusion bodies found in the plasma cells. They occur due to accumulation of immunoglobulins secreted by the plasma cells.

The two most characteristic histologic features found in biopsy are :

3. Levin test (complement fixation test) :High titres of antibodies against K. Rhinoscleromatis has been demonstrated. This indicates humoral immunity to be intact in these patients.

4. Radioimaging : MRI in the hypertrophic stage shows a soft tissue mass with mild to marked high signal intensity in both Tl- and T2-weighted images.

Treatment-Antibiotics : Both streptomycin (1 g/day) and tetracycline (2 g/day) for 4–6 weeks. Repeat if necessary after 1 month. Treatment is stopped only when two consecutive cultures from the biopsy material are negative.

Steroids : They can be combined to reduce fibrosis.

Surgery : In fourth stage of fibrosis and stenosis, surgery is required to establish the airway and correct nasal deformity. A silastic stent facilitates re-epithelialization.

Radiotherapy : It is not effective.

• Rifampicin :

Nasal Instillation - 1 Amp of rifampicin 125mg diluted in 20mL saline solution and 2mL of the solution is applied twice daily locally for a period of 8 weeks.Nasal Infiltration - 1 Amp of rifampicin 125mg infiltrated into granulomas every other day

• Kailasa Regime :

Carbolic acid (0.2ml) + Glacial acetic acid (0.2ml) + Glycerine (0.4ml) + 10ml distilled water is injected locally as 1 to 2 ml twice weekly at multiple sites of the lesion. This mixture causes chemical necrosis of granuloma.Usually 8-10 injections lead to complete regression of granuloma and restoration of normal nasal patency.

• Local application of 2 % acriflavin for a period of 8 weeks has been noted to be both efficacious and nontoxic.

(1) Acquired Syphilis : It occurs in three stages -

Primary syphilis Secondary syphilis Tertiary syphilis

1) Primary syphilis : It is also known as chancre. It is rarely seen at external nose or inside the vestibule. It appears as a hard, non painful ulcerated papule & is always associated with enlarged,

rubbery, and non-tender lymphadenopathy.A sore or chancre develops at the site of inoculation 10-90 days following infection.These lesions undergo spontaneous resolution within 6-10 weeks. It should be differentiated from malignant neoplasms and furunculosis.

• Malignant neoplasms usually affect older ages and undergoes relentless progression

• Furunculosis is a painful condition and it progresses to suppuration.

2) Secondary Syphilis :

Most infectious of all the three stages Symptoms 6-10 weeks after inoculation Secondary syphilis is rarely recognized in the nose, as mucous patches hardly

ever occur on such a thin, attenuated mucous membrane.The symptoms include:

Simple catarrhal rhinitis (persistent) Crusting & fissuring of nasal vestibule Mucous patches in the pharynx Roseolar / papular skin rashes Shotty, enlarged,non-tender lymph nodes. The scar of the primary lesion in the genitals or elsewhere may be visible.

3) Tertiary Syphilis :Only one-third of secondary syphilis cases progress to show clinical manifestations of tertiary

syphilis.This stage is commonly encountered in the nose. Lesion is also known as gumma.The lesion invades the mucous membrane, periosteum and bone.The bony portion of the nasal septum is frequently affected causing septal perforation. The symptoms include:

Pain / headache (worse during night) Swelling/obstruction of nose - swelling may be diffuse

or localized , is associated with offensive discharge, bleeding and crusting of the nose

Olfactory acuity diminishes Perforation of bony portion of nasal septum and

associated collapse of the bridge of the nose can cause structural damage to the nasal architecture (saddle nose deformity)

There may also be associated secondary atrophic rhinitis

DIAGNOSIS• It is made on the basis of serological tests.Serological

tests for syphilis include – VDRL, TPHA, FTA-ABS. The VDRL is a nontreponemal test and is relatively nonspecific but becomes positive early (four to five weeks) and correlates well with disease activity.

• Dark-field examination of moist or intentionally abraided dry lesions may demonstrate the organism.

• Lymph node aspirates or tissue specimens (lymph nodes, liver, skin or mucous membrane) may also be examined by silver stains or immunofluorescence for detection of spirochaetes

TREATMENT

• Penicillin is the drug of choice : benzathine penicillin 2.4 million units i.m. every week for 3 weeks with a total dose of 7.2 million units.

• Nasal crusts are removed by irrigation with alkaline solution.

• Bony and cartilaginous sequestra should also be removed.

• Local yellow mercury oxide ointment or Dilute mercuric nitrate ointment should be applied freely to the nasal vestibules.

• Cosmetic deformity is corrected after disease becomes inactive.

(2) Congenital syphilis -

Treponema pallidum pass from the infected mother to the fetus through the placenta.

The primary stage is not evident in Congenital syphilis.

Any of the lesions of secondary and tertiary forms of syphilis of nose can occur.

It classically begin during the 3rd week of life though it may appear around the 3rd month also,

Mucous batches may be seen on the nose, mouth, throat and larynx.

Radiating fissures form at the angle of mouth.

Blood-stained, thick, purulent nasal discharge block the nostrils and produce a bubbling sound during breathing termed as “syphilitic snuffles”.

Feeding problem results due to nasal obstruction.

Chronic rhinitis, septal perforation, palatal perforation is common.

Gummatous and destructive lesions occur most commonly at puberty.

Other stigmata of syphilis like Hutchinson's incisors, Moon's molars, interstitial keratitis and corneal opacities may be present

Nerve deafness can develop due to the involvement of the 8th cranial nerve endings. It presents as bilateral progressive loss of hearing which may become total.

Serologic tests for syphilis will invariably be negative

Management :

• In snuffles, the airway must be restored for feeding.• The nasal discharge is removed by gentle suction

and irrigation and the use of local drops of 0.5 percent ephedrine solution or simple normal saline, and by hyper extending the head before feeding.• In the tertiary forms, simple nasal toilet by

syringing with isotonic alkaline douche solution will remove the crusts and discharge.• Yellow mercuric oxide ointment may be applied

frequently to the nasal vestibules.

The nose is involved as a part of systemic disease, more often in the lepromatous than tuberculoid or dimorphous forms of disease.Nasal involvement in :

Tuberculoid Leprosy - Solitary skin lesions with cutaneous anaesthetic patches. There can be vestibule skin involvement, but there is no mucosal involvement.

LeprosyTuberculoid Leprosy Lepromatous Leprosy

Strong host resistanceNon-infectiveLocalized

Poor host resistanceInfectiveSystemic with widespread involvement of tissues

2 distinct polar forms of disease with the entire spectrum in between :

Lepromatous Leprosy -

There is skin, nerve & mucosal involvement Nasal mucosa scraping microscopy shows typical cigar pattern Lepra bacilli

Early Cases - Infection starts in the anterior part of nasal septum and anterior

end of inferior turbinate. Initially, there is excessive nasal discharge with red and swollen

mucosa. Later , there is

o nodular thickening of nasal mucosa (Nodules are paler than the surrounding mucosa with a yellowish tinge)

o crust formationo Blood stained discharge

Advanced cases - Nodular lesions on the septum may

ulcerate and cause perforation. Both the bony and cartilaginous

portions of nasal septum are destroyed due to perichondritis and periosteitis (nasal bridge collapse).

There can be sequelae like Atrophic Rhinitis, septal perforation & dorsal saddling.

Anterior nasal spine destruction is seen.

Radiographs showing the absence or erosion of anterior nasal spine are virtually diagnostic of Lepromatous leprosy.

Hyposmia is seen in 40% of cases.

Diagnosis : Microscopy of nasal discharge and nasal mucosa scrapings from the anterior end of

the inferior turbinate for acid-fast bacilli.Quantitative estimation of the bacterial count is obtained by noting the density of the bacilli in the smear which is recorded as the bacterial index. In addition, the morphology of the bacilli enables the observer to assess their viability.

Histology of nasal mucosa : Lepra cells seen.

Radiology: X-ray of paranasal sinuses in Waters view and lateral view reveals erosion of the anterior nasal spine and alveolar maxillary process and thick sinus mucosa.

General Treatment : Improve general hygiene. Regular nasal douching to clear the discharge and the crusts. Treat secondary infection.

Specific Treatment :• For multi-bacillary disease , the World Health Organization recommends a triple oral

drug-regimen of :• rifampin, 600 mg once a month; • dapsone, 100 mg daily; and • clofazimine, 300 mg once a month and 50 mg daily for 12 months,

• For pauci-bacillary disease, the recommendation is :• rifampin, 600 mg once a month, and• dapsone, 100 mg daily for 6 months.

Primary tuberculosis of nose is rare. More often it is secondary to lung tuberculosis.

Anterior part of nasal septum and anterior end of inferior turbinate are the sites commonly involved.

First, there is nodular infiltration followed later by ulceration and perforation of nasal septum in its cartilaginous part.

Tuberculosis

Symptoms -• Nasal discharge• Nasal obstruction• Pain• Epistaxis• H/O haemoptysis• Evening rise of temperature• Cough

Signs -• Bright, red nodular thickening of septum with or without ulceration, bilaterally• Septum—Cartilaginous part is affected.• Septum—Ulceration, perforation• Fibrosis seen in late stages

Lupus vulgaris (also known as Tuberculosis luposa) are painful cutaneous tuberculosis skin lesions with nodular appearance, most often on the face around the nose, eyelids, lips, cheeks, ears and neck.

Indolent and Chronic from of Tuberculosis infection F:M = 2:1 (young adults)It is mostly prevalent in Temperate Climate.Starts at Vestibule, extends to adjoining skin and mucosa.Most common location is the mucocutaneous junction.

Lupus vulgaris

Symptoms• Nasal obstruction• Nasal discharge (foul smelling)• Epistaxis• Deformity of the nose

Signs - • Early stage—reddish, firm nodule• Late stage—superficial ulceration• Crusting• Butterfly appearance• Ulceration followed by fibrosis• Perforation of the cartilaginous part of the nasal sep tum

Complications :

• Pulmonary tuberculosis may develop• Dacryocystitis• Nasopharyngeal lupus• Atrophic rhinitis• Epithelioma in the infected tissue• Lupus of the face and nasopharynx• Malignant transformation

Investigations –

• Blood examination• Bacteriological examination of the discharge may show tubercle

bacilli• Biopsy—Typical tubercle with sparse caseation necrosis• Chest X Ray—Pulmonary tuberculosis may develop• Mantoux test—strongly positive• Diascopy –

o Diascopy is a test for blanchability performed by applying pressure with a finger or glass slide and observing color changes.

o In lupus, upon diascopy, thr reddish brown nodule become more prominent by contrast— Apple jelly nodules

Treatment :

• Anti-tubercular treatment therapy for 6-9 months.• Improve general hygiene.• Regular nasal douching to clear the discharge and the crusts.• Calciferol (Vitamin Da) I ,50,000 units daily for 6-9 months• Reconstructive surgery of the residual nasal defor mity after arrest of

the disease.