Otolaryngological manifestations of systemic diseases

1. Otolaryngological manifestations of
systemic diseases
Dr. Krishna Prasad Koirala
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2. Otolaryngological manifestations of :
–HIV
–Tuberculosis
–Sarcoidosis
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3. HIV/AIDS IN ENT
• Caused by retrovirus of lentivirus subfamily
• Up to 80% of HIV-infected patients eventually develop ENT manifestations
• Oral disease appears to be the most common
• Cause: immunodeficiency leading to
– Opportunistic infections by viruses, fungi, bacteria, protozoa etc.
– Activation of neoplastic process e.g, Kaposi's sarcoma, Non-Hodgkin
lymphoma
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4. •Predisposing factors for HIV-related ENT conditions
–CD4+ cell count of less than 200/µL
–Plasma HIV-RNA levels greater than 3000 copies/mL
–Xerostomia
–Poor oral hygiene
–Smoking
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5. • Oro-pharyngeal :
– Candidiasis, periodontal and gingival disease, HSV and HPV infection, oral hairy
leucoplakia, Kaposi's sarcoma, non-Hodgkin's lymphoma.
• Neck :
– Cervical lymphadenopathy, parotid gland enlargement
• Nose and PNS: sinusitis (often due to atypical bacteria), allergic rhinitis
• Ear:
– Otitis externa, otitis media, inner ear involvement (sensorineural hearing loss,
disequilibrium), facial nerve palsy
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6. Oral Cavity - Fungal infection
• Oral Candidiasis
− Most frequently opportunistic infection in HIV
− Approx. 90% of patients affected
• Clinical patterns
– Pseudomembranous : creamy, white, curd-like plaques on the buccal
mucosa, tongue, and other oral mucosal surfaces. Can be wiped out leaving a
bleeding base
– Erythematous : red, flat, subtle lesion on the dorsal surface of the tongue,
or on the hard or soft palate
– Angular cheilitis: Fissure & ulcer at oral commissure
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7. • Diagnosis: Clinical
–Staining : KOH mount , Gimsa stain, Periodic Acid Schiff stain
–Biopsy : to r/o malignancy
• Treatment
–Amphotericin, nystatin, clotrimazole locally for mild cases
–Fluconazole, ketoconazole, voriconazole systemic for moderate to
severe cases
(Minimum of two weeks to reduce the colony forming units to the lowest level possible and prevent recurrence)
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8. Oral cavity : Bacterial infections
• Periodontal diseases
– With CD4 count <100
• 2 types
− Linear gingival erythema
− Necrotizing gingivitis/ ulcerative periodontitis
• Treatment:
– Debridement, betadine/chlorhexidine gargle,
metronidazole
• Oral and oropharyngeal tuberculosis
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9. Oral cavity : Viral infections
Herpes simplex (Occurs in up to 9% of adults)
• Intraoral :
– Small rounded ulcers with erythematous halo in hard
palate , gingiva and dorsum of tongue (often bigger, recur
more frequently, and tend to be more persistent in HIV infected patients)
• Extraoral - Herpes labialis
– Commonest manifestation, fever blister
– More numerous , lasts longer and reoccur faster
• Diagnosis : Tzank test – monoclonal anti HSV Ab
• Treatment : acyclovir
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10. • Oral Hairy Leukoplakia
– Caused by EBV and is one of the first opportunistic
infections in HIV-positive patients
– White corrugated lesion on the lateral border of the
tongue that cannot be scraped (appear hairy due to
elongated filiform papillae)
– Treatment : Topical trichloroacetic/glycolic acid,
podophyllum, oral acyclovir, zidovudine and sulfa drugs
• Oral Human Papilloma Virus
• Warty or papillomatous lesions in the mucous membrane
of oral cavity and oropharynx
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11. Oral Cavity - Neoplastic lesions
• Kaposi's sarcoma
– Multifocal neoplasm of vascular endothelial origin
(oral, skin and visceral involvement)
– Intraoral lesion may be the first symptom of late
stage HIV disease
– Can be macular, nodular, or raised and ulcerated
– Most common in hard palate followed by gingival and
buccal mucosa , soft palate, and dorsum of tongue
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12. • O/ E : Flat, reddish areas that do not blanch, can enlarge,
ulcerate or get infected, pain and bleeding is common
• Diagnosis : biopsy
– HPE: interweaving bundles of spindle shaped cells with
vascular slits & RBC extravasations
• Treatment :
− Surgery: local excision or electrodesiccation and
curettage, cryosurgery
− Radiation therapy
− Chemotherapy : vincristine, liposomal doxorubicin
− Biologic therapy : interferon alfa
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13. • Non - Hodgkin’s lymphoma
– AIDS defining criteria
– Second most common malignancy in HIV infected
individuals
– B cell in origin, occurs as focal, ulcerated soft tissue
mass on the palate or gingival tissues
– Occur in 10-30% of AIDS patients
– Localized nodal or extranodal disease
– Occurs after CD4 count < 200/ mm3
– Firm painless /painful swelling with or without ulcer
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14. Neck manifestations in HIV/ AIDS
• Cervical lymphadenopathy
–Most common manifestation of HIV infection in the neck
– Causes : Reactive lymphadenitis, tuberculosis, lymphoma, Kaposi's
sarcoma
–Presence of diffuse lymphadenopathy in two or more sites of the
neck for longer than three months (HIV lymphadenopathy)
–Soft and symmetrical , mostly in the posterior triangle
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15. • Salivary gland diseases
–Parotid swelling:
• Bilateral, occasionally cystic
–Causes:
• Reactive hyperplasia of an intraparotid lymph node
• Benign lymphoepithelial lesions with ductal metaplasia
• Benign lymphoepithelial cysts
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16. HIV –EAR manifestations
• Seborrhic dermatitis: Usually preauricular
• Otitis externa, otomycosis, Pneumocystis carinii infected aural polyps
• Serous otitis media : Frequent URTI, lymphoid hyperplasia, adenoid
hypertrophy, nasopharyngeal tumor
• AOM, Kaposi's sarcoma of EAC
• Sudden SNHL: Unilateral/Bilateral CMV, ototoxic drugs, demyelination,
• Facial paralysis
• Activation of latent syphilis: otosyphilis
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17. HIV manifestations in nose
• HSV infection
• Allergic rhinitis : increased Ig E level & eosinophils
• Chronic rhinosinusitis with nasal crusting & dryness
• Acute fungal sinusitis
• Nasal and nasopharyngeal tumours
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18. Sinusitis
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19. HIV Manifestations in larynx and esophagus
• Epiglottitis, Kaposi's sarcoma, HSV, CMV, Candidiasis
• Histoplasmosis, Coccidiomycosis, aspergillosis, candidiasis
• Three categories of respiratory tract infection s/o immunodeficiency
– Acute overwhelming respiratory infection
– Acute episodes of infection with normal intervening period
(recurrent pneumonia , bronchial infection)
– Chronic purulent bronchial disease, purulent sinusitis
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20. Tuberculosis – Ear manifestations
• In association or secondary to pulmonary tuberculosis
• Infection reaches to middle ear through Eustachian tube
• Painless watery otorrhea not responding to conventional
treatment , multiple perforations in TM and pale
granulations (chicken fat) are common findings
• Complications
– Mastoiditis, facial palsy, labyrinthitis, SNHL
• Treatment : Systemic antitubercular therapy
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21. Nasal manifestations in tuberculosis
• Rare affection, can be primary or secondary to
pulmonary tuberculosis
• Lesions can be ulcerative, infiltrative, or
proliferative and mostly unilateral in the
cartilaginous nasal septum
• Presents as nasal stuffiness (nasal obstruction),
epistaxis, nasal discharge, crusting, recurrent
nasal polyps, and ulcers
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22. Lupus Vulgaris
• Chronic indolent post primary infection of mycobacterium tuberculosis
• Common site - Membranous and cartilaginous junction of nasal septum, trauma?
• Clinical features
− Butterfly appearance of the facial skin
− Nasal obstruction, foul smell, crusting ,epistaxis
− Ulceration and fibrosis leading to distortion of nasal ala, tip, vestibule
− Cartilaginous septal perforation
− Diascopy makes the reddish-brown nodule more evident (Apple jelly Nodule)
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23. Pharyngeal tuberculosis
• Multiple painful shallow ulcers in the pharynx and oral cavity
• Primary
–Adenoids/tonsils in children
–Infected by contaminated cow’s milk in the past ( bovine TB)
• Secondary
–Secondary to coughing up heavily infected sputum
–Blood born in cases of miliary tuberculosis
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24. Laryngeal tuberculosis
• Almost always secondary to pulmonary TB
• Sputum positive rate : 90-95%
• Spread
–Bronchogenic following contact of sputum containing AFB
–Rarely hematogenous or lymphatic spread
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25. Pathology of laryngeal tuberculosis
• Stages:
– Exudation + hyperaemia : subepithelial inflammation
– Infiltration : subepithelial space infiltration causing pseudo - edema
– Proliferation or granuloma formation : Tubercle formation (characteristic lesion),
Coagulation necrosis with central caseation
– Ulceration : tubercles break down to form superficial shallow ulcers with
undermined margins which might progress to cartilage : perichondritis (very painful
condition)
– Cicatrization : Healing of ulcer by fibrosis
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26. • Clinical features
–Women - more commonly affected
–Hoarseness, productive cough, evening rise of temperature with chills,
–Odynophagia (due to shallow ulcers with exposed nerve ending)
• O/E:
– Laryngoscopy - Redness & edema of the cord with normal appearing
opposite cord
– Stroboscopy shows decreased amplitude & edge movements &
possible phonatory arrest
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28. Sarcoidosis
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29. • Sarcoidosis is defined as a rare systemic granulomatous disease of
unknown etiology with a non-caseating hard granulomatous
inflammation
• Usually involves : Lungs, parotid gland , facial nerve, nasal cavity ,larynx,
nervous system
• Etiology:
– Special form of TB
– Undifferentiated organism
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30. • Hearing loss
– Sudden, fluctuating or progressive SNHL
– B/L asymmetrical
– Caloric test - no response
• Uveitis ~ 80%
• Parotid swelling ~20%
• Facial Nerve Palsy~43%
• Lymphadenopathy~55%
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31. Signs in the nose
• Mucosal – commonest :
Yellow nodule surrounded by hyperemic boggy granular
mucosa in anterior septum and inferior turbinates leading
to adhesions and stenosis of anterior nares
• Skin
• Lupus Pernio: resemblance of perniosis or cold induced injury
(Mortimer’s malady) symmetrical, bulbous, bluish red, dusky,
glistening lesion d/t stretching epidermis & large pilosebaceous
follicle
• Small lesions affect- cheek, lips, finger, & ear (Turkey ear)
• Nasal bone: swollen nasal bridge5/11/2020 at 11:00 AM 31

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33. Larynx
• 10% involvement
• Hoarseness, dysphagia, dyspnea
• Epiglottis and false cord are swollen, edematous and pale
• Rim of epiglottis full & rounded
• True cord and subglottis rarely affected
• Lesion progresses rapidly causing life threatening airway obstruction
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34. Investigations
• Chest X-Ray:
– B/L hilar LN enlargement, parenchymal infiltrates, fibrosis
• Biopsy:
• Non caseating granuloma (hard)
• Underdeveloped surrounding rim of lymphoid cells (naked
tubercle)
• Calcium and protein inclusions inside the Langhans giant
cells (Schaumann bodies)
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35. • Kveim- Siltzbach test
– Intradermal injection of filtered extract of spleen from a case
of sarcoidosis  skin biopsy 6 wks later development of
nodule in all mucosal cases and 75 -90% of active
sarcoidosis
• Serum ACE ( Angiotensin Converting Enzyme) :
– Increased in 60% of active diseases (produced by
epithelioid cells derived from activated macrophage)
• Serum/urine calcium : to exclude hypercalcemia (uncontrolled
synthesis of 1,25-dihydroxyvitamin D3 by macrophages)
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36. Non-specific treatment
• Steroids - Topical & Systemic
– Prednisolone - 1mg/kg/day X 4-6 wks - taper over 2-3 months
– Effective in mucosal disease but ? in systemic disease
• Chloroquine: 250 mg PO alternate days X 9 months
– Used in combination in patients not responding to steroid
• Methotrexate 5mg PO weekly X 3 mths (alternative)
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37. Syphilis
• Late Syphilis
– Affects the temporal bone 10-
50 years after the onset of
primary infection
– Hearing loss is permanent with
fluctuation in hearing
– Female >male
• Early Syphilis
– Malaise, Pyrexia, Headache, Skin
eruptions
– Pharyngitis, Lymphadenopathy
– Sudden B/L hearing loss - High
frequency SNHL
– Decreased Stapedial reflex
– Decreased Caloric response
– Endolymphatic hydrops
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38. • PTA : flattened or high frequency SNHL
• Stapedial reflex : Alteration to retrocochlear pattern
• Decreased speech discrimination
• Progressive severe peripheral vestibular damage: increased
imbalance + ataxia
• Positive fistula test without middle ear disease (Henebert’s sign)
• Tullio’s phenomenon : transient vertigo & nystagmus on exposure to
sudden high intensity sound
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39. • Primary Syphilis
• Lesion: CHANCRE, appears in external nose or inside the vestibule
• Hard, non painful, ulcerated papule appears after 3-4 wks of contact
• Disappears spontaneously in 6-10 wks
• LN - Enlarged, rubbery ,non tender
• Secondary syphilis
• Most infectious stage
• Commonest presentation- Simple catarrhal rhinitis with crusting/fissuring of nasal
vestibule
• Mucous patches, roseolar papular rashes, pyrexia, shotty enlarged LN
Nasal Syphilis
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40. Tertiary Syphilis
• Tenderness on the bridge of nose - characteristic sign
• Pain & headache (worsens at night)
• Lesion: GUMMA
• Bony septal perforation most common: leads to saddle nose
• Hyposmia/Anosmia, offensive discharge, crusting & bleeding are common
• Bare bone may be felt with the probe when crust is removed
• Severe scarring leads to secondary atrophic rhinitis
• Scarring & stenosis of nasal passage
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41. Oral cavity and oropharynx
• Primary: Chancre of lips, buccal mucosa, tongue & tonsils in descending order
• Secondary:
– Snail track ulcer or mucous patches covered with greyish white membrane which
on scrapping has pink base but no bleeding
• Tertiary:
– Gumma of hard palate, tonsils , posterior pharyngeal wall leading to oronasal
and oroantral fistula
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43. Larynx
• Affected in only secondary or tertiary stage
• Hoarseness, dysphagia or stridor
• Lesions have predilection on anterior part of larynx, epiglottis &
aryepiglottic fold in contrast to tuberculosis
• Secondary : Erythematous mucous patches, greyish lesion
• Tertiary : Ulcers, granulomas, fibrosis, Laryngeal stenosis
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