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Granulomatous Diseases of the Head and Neck

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Title: Granulomatous Diseases of the Head and Neck


1
Granulomatous Diseases of the Head and Neck
  • Sarah Rodriguez, MD
  • Byron Bailey, MD

2
Granulomatous Inflammation
  • A type of chronic inflammation characterized by
    concentric layers of cells consisting of
    specialized macrophages called epithelioid cells
    and multinucleated giant cells surrounded by
    lymphocytes and fibroblasts however, the
    inflammatory process can be more diffuse without
    discrete classic granuloma formation
  • Stimulus can be foreign body or persistent
    microorganism which evades destruction multiple
    etiologies exist

3
Outline
  • Infectious
  • Bacterial
  • Fungal
  • Parasitic
  • Trauma/Foreign Body
  • Neoplastic
  • Inflammatory Disease of Unknown Etiology
  • Autoimmune/Vasculitic Disease

4
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5
Cat Scratch Disease and Bacillary Angiomatosis
  • Bartonella henselae is most common organism
  • CSD usually self-limited of children and young
    adults requiring no specific treatment
  • BA occurs in immunocompromised patients and
    requires antibiotic treatment visceral
    involvement termed peliosis hepaticus

6
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7
Rhinoscleroma
  • Organism Klebsiella rhinoscleromatis
  • Three stages catarrhal, granulomatous, sclerotic
  • Treatment is with tetracycline or cipro
  • Nasal involvement universal paranasal sinus
    involvement much less common

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9
Leprosy (Hansens Disease)
  • Organism Mycobacterium leprae
  • Patterns of manifestation include lepromatous,
    tuberculoid and mixed
  • Rare in United States
  • Treatment is with 2-5 years of antibiotics

10
Nontuberculous Mycobacteria
  • Pathogens include M. scrofulaceum, M. avium
    complex, M. kansasii, M. marinum
  • Many manifestations commonly lymphadenitis in
    children or severe systemic infection in
    immunocompromised

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12
Tuberculosis
  • Extrapulmonary tuberculosis most commonly affects
    infants, children and immunocompromised.
  • Most common extrapulmonary manifestation is
    scrofula

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14
Actinomycosis
  • Cervicofacial disease most commonly secondary to
    dental infection, intraoral trauma or
    manipulation
  • Characteristic sulfur granules classically
    perimandibular soft tissue infection or abscess
    with draining sinus and possible bone involvement
  • Requires anaerobic processing of culture
    specimens
  • Can be treated with ampicillin, Pen G or
    Clindamycin

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16
Syphilis
  • Organism spirochete Treponema pallidum
  • Three stages primary, secondary, tertiary
  • Transmitted sexually or transplacentally
  • Treatment is with penicillin

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18
Histoplasmosis
  • Organism is Histoplasma capsulatum
  • Risk factor is exposure to soil enriched with bat
    or bird excrement
  • Manifestation of infection depends on number of
    organisms inhaled and immune status
  • May cause mediastinal granulomatosis and
    fibrosing mediastinitis
  • Treatment is with Amphotericin B or Itraconazole
    depending on severity of disease

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20
Candidiasis
  • Many different Candida species can be pathogenic
  • Can cause oral thrush or candida esophagitis
  • Candida may also be part of normal flora of oral
    cavity
  • Treatment is usually via topical therapy
    systemic therapy may be required with severe
    infection or immunocompromised patients

21
Blastomycosis
  • Blastomyces dermatitidis dimorphic fungus found
    in moist soil
  • Extrapulmonary cutaneous disease usually occurs
    in conjunction with pulmonary disease
  • May involve multiple organ systems especially in
    immunocompromised patients

22
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23
Coccidiomycosis
  • Coccidiodes immitis is a fungus that lives in
    dry, desert soil
  • Infection is via inhalation of arthrospores
  • Can be asymptomatic or lead to pulmonary
    infection which can be severe
  • May manifest as skin lesion in which case
    pulmonary or CNS involvement should be suspected
  • Treatment is via Amphotericin B or one of the
    azoles

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25
Phycomycosis
  • Sinonasal mucormycosis with etiologies including
    Aspergillus sp. or organisms from the family
    Mucorales Rhizopus, Rhizomucor, Mucor, Absidia
    and Cunninghamella
  • Susceptible patients are immunocompromised
    especially patients with DKA
  • Spectrum of disease ranging from rapidly
    progressive and fatal to indolent invasive course
  • Symptoms include facial anesthesia, headache,
    ophthalmoplegia, facial necrosis and obtundation
  • Physical exam may reveal white, insensate nasal
    mucosa, black necrotic mucosa changes prominent
    on middle turbinate, hard palate
  • Treatment is with amphotericin B and urgent and
    aggressive debridement

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27
Leishmaniasis
  • The bite of a sandfly carrying various leishmania
    species is the etiology of leishmaniasis
  • Visceral (kala-azar), cutaneous, and
    mucocutaneous (Espundia)
  • Treatment is quite toxic

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29
Myiasis
  • Infestation with maggots of screw worm or bot fly
  • Furuncular, creeping dermal myiasis
  • Treatment consists of surgical debridement

30
Infectious Etiology
  • Bacterial
  • Cat Scratch Disease
  • Rhinoscleroma
  • Leprosy
  • Nontuberculous Mycobacteria
  • Tuberculosis
  • Actinomycosis
  • Syphilis
  • Fungal
  • Histoplasmosis
  • Candidiasis
  • Blastomycosis
  • Coccidiomycosis
  • Phycomycosis
  • Parasitic
  • Leishmaniasis
  • Myiasis

31
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32
Intubation Granuloma
  • Almost universally involves vocal process of
    arytenoid
  • Hoarseness and foreign body sensation common
  • Voice rest, control of irritant exposure,
    possible surgical excision

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34
Teflon Granuloma
  • 2-3 of patients receiving Teflon for unilateral
    vocal cord paralysis develop increasing dysphonia
    or even airway obstruction secondary to
    hyperintense granulomatous response to Teflon
  • Treatment is via endoscopic removal, laser
    vaporization or open surgical removal

35
Trauma/Foreign Body
  • Intubation Granuloma
  • Teflon Granuloma

36
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37
Langerhans Cell Histiocytosis
  • Formerly included
  • Eosinophilic granuloma (usually monostotic
    osteolytic lesion with predilection for skull)
  • Hand-Schuller-Christian disease (classic triad of
    multiple bone lesions, exophthalmos and diabetes
    insipidus)
  • Letterer-Siwe disease (acute, disseminated)

38
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39
Lobular Capillary Hemangioma (Pyogenic Granuloma)
  • Neither infectious or granulomatous
  • Exact etiology unknown
  • Solitary glistening red papule prone to bleeding
    and ulceration
  • Occurs most often on trunk, head, neck, upper
    extremities
  • Oral cavity lesion may develop in pregnancy
    (pregnancy tumor)

40
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41
Necrotizing Sialometaplasia
  • Non-neoplastic condition of the salivary gland,
    typically the minor salivary glands of the palate
  • Is self-limiting
  • Etiology unknown but may be related to trauma or
    vomiting
  • Typically an ulcerative lesion on the hard palate
  • Can be confused with squamous cell carcinoma and
    mucoepidermoid carcinoma on histopathologic
    evaluation

42
Neoplastic
  • Langerhans Cell Histiocytosis
  • Lobular Capillary Hemangioma
  • Necrotizing Sialometaplasia

43
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44
Sarcoidosis
  • In the United States, the disease is more
    prevalent in African Americans slight female
    preponderance
  • Disease manifestations and course variable
    pulmonary involvement almost universal
  • Lofgrens syndrome hilar lymphadenopathy and
    erythema nodosum Heerfordts syndrome
    (uveoparotid fever) anterior uveitis/parotid
    swelling/facial nerve palsy/fever
  • Otolaryngologic manifestations occur in 10-15 of
    patientscervical adenopathy, parotid swelling
    and facial nerve palsy are the most common
    findings
  • High rate of spontaneous remission
  • Diagnosis via BAL with high CD4/CD8 ratio and
    transbronchial lung biopsy
  • Elevated ACE suggestive but may not be present
  • Treatment via steroids/methotrexate

45
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46
Idiopathic Midline Destructive Disease
  • Rare spectrum of lymphoproliferative disorders
    causing destruction of the nose, paranasal
    sinuses, palate and facial soft tissue
  • Controversy exists over whether all cases of IDMM
    are lymphoma or Wegeners
  • Treatment is with XRT

47
Inflammatory Diseases of Unknown Etiology
  • Sarcoidosis
  • Idiopathic Midline Destructive Disease

48
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49
Wegeners Granulomatosis
  • Classically the triad of pulmonary, renal, and
    head and neck manifestations
  • Nose and paranasal sinuses most commonly affected
    site in the head and neck
  • Diffuse crusting of the nose and nasopharyx
    removal of crusts leaves friable mucosa
  • Orbital involvement common nasolacrimal duct
    obstruction/proptosis due to pseudotumor
  • Subglottic involvement apparent in 1/5 of
    patients
  • Diagnosis involves serum ANCA testing and biopsy
    (remove all crusts and biopsy every turbinate)
  • Treatment is with steroids and cyclophosphamide
    with long term bactrim

50
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51
Relapsing Polychondritis
  • Patients with this rare disorder produce
    antibodies to type II collagen
  • Ear exam in acute case may reveal red, swollen
    and tender ear with sparing of the lobule over
    time, the ear becomes droopy
  • Nasal chondritis may lead to saddle nose
    deformity
  • 50 of patients have laryngotracheal disease with
    potential dynamic collapse of the trachea

52
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53
Sjogrens Syndrome
  • Affects salivary and lacrimal glands
  • May be primary (sicca syndrome) or secondary
  • (in association with other autoimmune diseases)
  • May evolve into lymphoma
  • 90 patients women
  • Monitor for signs of lymphoma
  • Check for other autoimmune diseases
  • SS-A, SS-B antibodies

54
Autoimmune/Vasculitic Disorders
  • Wegeners Granulomatosis
  • Relapsing Polychondritis
  • Sjogrens Syndrome

55
Bibliography(Photo Sources)
  • Color Atlas and Synopsis of Clinical Dermatology
    4th Ed. Fitzpatrick, Johnson, Wolff and Suurmond.
  • Mount Allison University Website, from the Mount
    Allison Science Image Collection
  • A Colour Atlas of Infectious Diseases 2nd ed.
    Emond and Rowland.
  • A Color Atlas of Otorhinolaryngology. Benjamin,
    Bingham, Hawke, Stammberger. 1995
  • Otolaryngology Head and Neck Surgery. Current
    Concepts of the Lethal Midline Granuloma
    Syndrome. 100(6) 623-630
  • Annals of Otology, Rhinology and Laryngology.
    Lateral Laryngotomy for the Removal of Teflon
    Granuloma. 107735-744 and Rhinoscleroma
    Mimicking Nasal Polyposis 110290-292
  • A Pocket Guide to Fungal Infection. Richardson
    and Johnson. 2000
  • Infectious Diseases. Farrar, Wood, Innes, Tubbs.
    2nd ed. 1992
  • Atlas of Infectious Diseases. Stone, Gorbach.
    2000.
  • Diagnostic Laryngology Adults and Children.
    Benjamin. 1990
  • Tropical Medicine and Parasitology 5th ed. Peters
    and Pasvol. 2002
  • Otolaryngologic Clinics of North America.
    Mucormycosis of the Nose and Paranasal Sinuses
    B. J. Ferguson. 33(2) Apr 2002
  • Laryngoscope. Necrotizing Sialometaplasia. J.
    Gavron, J. Ardito, A. Curtis. July 1981 Vol 91
  • Kelleys Textbook of Rheumatology 6th ed. Ruddy.
    2001
  • Textbook of Primary Care Medicine 3rd ed. Noble.
    2001
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