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Granuloma-slides-2003-1029 - Granulomatous Diseases of the...

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Unformatted text preview: Granulomatous Diseases of the Head and Neck the Sarah Rodriguez, MD Byron Bailey, MD Granulomatous Inflammation Granulomatous A type of chronic inflammation characterized by type 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 discrete Stimulus can be foreign body or persistent Stimulus microorganism which evades destruction; multiple etiologies exist etiologies Outline Outline Infectious Infectious – Bacterial Bacterial – Fungal – Parasitic Trauma/Foreign Body Neoplastic Inflammatory Disease of Unknown Etiology Autoimmune/Vasculitic Disease Cat Scratch Disease and Bacillary Angiomatosis Angiomatosis Bartonella henselae is most common organism most CSD usually self-limited of CSD children and young adults requiring no specific treatment treatment BA occurs in BA immunocompromised patients and requires antibiotic treatment; visceral involvement termed peliosis hepaticus termed Rhinoscleroma Rhinoscleroma Organism: Klebsiella Organism: rhinoscleromatis rhinoscleromatis Three stages: catarrhal, Three granulomatous, sclerotic granulomatous, Treatment is with Treatment tetracycline or cipro tetracycline Nasal involvement Nasal universal; paranasal sinus involvement much less common common Leprosy (Hansen’s Disease) Leprosy Organism: Organism: Mycobacterium leprae Mycobacterium Patterns of Patterns manifestation include lepromatous, tuberculoid and mixed tuberculoid Rare in United States Treatment is with 2-5 Treatment years of antibiotics years Nontuberculous Mycobacteria Nontuberculous Pathogens include M. Pathogens scrofulaceum, M. avium complex, M. kansasii, M. marinum kansasii, Many manifestations; Many commonly lymphadenitis in children or severe systemic infection in immunocompromised immunocompromised Tuberculosis Tuberculosis Extrapulmonary Extrapulmonary tuberculosis most commonly affects infants, children and immunocompromised. Most common Most extrapulmonary manifestation is scrofula scrofula Actinomycosis Actinomycosis Cervicofacial disease most commonly secondary Cervicofacial to dental infection, intraoral trauma or manipulation manipulation Characteristic sulfur granules; classically Characteristic perimandibular soft tissue infection or abscess with draining sinus and possible bone involvement with Requires anaerobic processing of culture Requires specimens specimens Can be treated with ampicillin, Pen G or Can Clindamycin Clindamycin Syphilis Syphilis Organism: spirochete Treponema pallidum Organism: Treponema Three stages: primary, secondary, tertiary Transmitted sexually or transplacentally Treatment is with penicillin Histoplasmosis Histoplasmosis Organism is Histoplasma capsulatum Organism Histoplasma Risk factor is exposure to soil enriched with bat or Risk bird excrement bird Manifestation of infection depends on number of Manifestation organisms inhaled and immune status organisms May cause mediastinal granulomatosis and May fibrosing mediastinitis fibrosing Treatment is with Amphotericin B or Itraconazole Treatment depending on severity of disease depending Candidiasis 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 Candida cavity cavity Treatment is usually via topical therapy; systemic Treatment therapy may be required with severe infection or immunocompromised patients Blastomycosis Blastomycosis Blastomyces dermatitidis: dimorphic fungus found in moist soil moist Extrapulmonary cutaneous Extrapulmonary disease usually occurs in conjunction with pulmonary disease pulmonary May involve multiple organ May systems especially in immunocompromised patients patients Coccidiomycosis Coccidiomycosis Coccidiodes immitis is a fungus that lives in dry, Coccidiodes desert soil desert Infection is via inhalation of arthrospores Can be asymptomatic or lead to pulmonary Can infection which can be severe infection May manifest as skin lesion in which case May pulmonary or CNS involvement should be suspected suspected Treatment is via Amphotericin B or one of the Treatment azoles azoles Phycomycosis Phycomycosis Sinonasal mucormycosis with etiologies including Aspergillus sp. Sinonasal Aspergillus or organisms from the family Mucorales: Rhizopus, Rhizomucor, Mucor, Absidia and Cunninghamella Mucor, Susceptible patients are immunocompromised especially Susceptible patients with DKA patients Spectrum of disease ranging from rapidly progressive and fatal Spectrum to indolent invasive course to Symptoms include facial anesthesia, headache, Symptoms ophthalmoplegia, facial necrosis and obtundation ophthalmoplegia, Physical exam may reveal white, insensate nasal mucosa, black Physical necrotic mucosa; changes prominent on middle turbinate, hard palate palate Treatment is with amphotericin B and urgent and aggressive Treatment debridement debridement Leishmaniasis Leishmaniasis The bite of a sandfly carrying various The leishmania species is the etiology of leishmaniasis leishmaniasis Visceral (kala-azar), cutaneous, and Visceral mucocutaneous (Espundia) Treatment is quite toxic Myiasis Myiasis Infestation with Infestation maggots of screw worm or bot fly worm Furuncular, creeping Furuncular, dermal myiasis dermal Treatment consists of Treatment surgical debridement surgical Infectious Etiology Infectious Bacterial – – – – – – – Fungal – – – – – Cat Scratch Disease Rhinoscleroma Leprosy Nontuberculous Mycobacteria Tuberculosis Actinomycosis Syphilis Histoplasmosis Candidiasis Blastomycosis Coccidiomycosis Phycomycosis Parasitic – Leishmaniasis – Myiasis Intubation Granuloma Intubation Almost universally involves vocal process of Almost arytenoid arytenoid Hoarseness and foreign body sensation Hoarseness common common Voice rest, control of irritant exposure, Voice possible surgical excision possible Teflon Granuloma Teflon 2-3% of patients receiving Teflon for 2-3% unilateral vocal cord paralysis develop increasing dysphonia or even airway obstruction secondary to hyperintense granulomatous response to Teflon granulomatous Treatment is via endoscopic removal, laser Treatment vaporization or open surgical removal vaporization Trauma/Foreign Body Trauma/Foreign Intubation Granuloma Teflon Granuloma Langerhan’s Cell Histiocytosis Langerhan’s Formerly included: – Eosinophilic granuloma (usually monostotic Eosinophilic osteolytic lesion with predilection for skull) osteolytic – Hand-Schuller-Christian disease (classic triad of Hand-Schuller-Christian multiple bone lesions, exophthalmos and diabetes insipidus) diabetes – Letterer-Siwe disease (acute, disseminated) Lobular Capillary Hemangioma (Pyogenic Granuloma) (Pyogenic Neither infectious or granulomatous Exact etiology unknown Solitary glistening red papule prone to Solitary bleeding and ulceration bleeding Occurs most often on trunk, head, neck, Occurs upper extremities upper Oral cavity lesion may develop in pregnancy Oral (“pregnancy tumor”) (“pregnancy Necrotizing Sialometaplasia Necrotizing Non-neoplastic condition of the salivary gland, Non-neoplastic typically the minor salivary glands of the palate typically Is self-limiting Etiology unknown but may be related to trauma or Etiology vomiting vomiting Typically an ulcerative lesion on the hard palate Can be confused with squamous cell carcinoma Can and mucoepidermoid carcinoma on histopathologic evaluation histopathologic Neoplastic Neoplastic Langerhan’s Cell Histiocytosis Lobular Capillary Hemangioma Necrotizing Sialometaplasia Sarcoidosis Sarcoidosis In the United States, the disease is more prevalent in African In Americans; slight female preponderance Americans; Disease manifestations and course variable; pulmonary involvement Disease almost universal almost Lofgren’s syndrome: hilar lymphadenopathy and erythema nodosum; Lofgren’s Heerfordt’s syndrome (uveoparotid fever): anterior uveitis/parotid swelling/facial nerve palsy/fever swelling/facial Otolaryngologic manifestations occur in 10-15% of patients—cervical Otolaryngologic adenopathy, parotid swelling and facial nerve palsy are the most common findings common High rate of spontaneous remission Diagnosis via BAL with high CD4/CD8 ratio and transbronchial lung Diagnosis biopsy biopsy Elevated ACE suggestive but may not be present Treatment via steroids/methotrexate Idiopathic Midline Destructive Disease Disease Rare spectrum of lymphoproliferative Rare disorders causing destruction of the nose, paranasal sinuses, palate and facial soft tissue tissue Controversy exists over whether all cases of Controversy IDMM are lymphoma or Wegener’s Treatment is with XRT Inflammatory Diseases of Unknown Etiology Etiology Sarcoidosis Idiopathic Midline Destructive Disease Wegeners Granulomatosis Wegeners Classically the triad of pulmonary, renal, and head and Classically neck manifestations neck Nose and paranasal sinuses most commonly affected site Nose in the head and neck in Diffuse crusting of the nose and nasopharyx; removal of Diffuse crusts leaves friable mucosa crusts Orbital involvement common: nasolacrimal duct Orbital obstruction/proptosis due to pseudotumor obstruction/proptosis Subglottic involvement apparent in 1/5 of patients Diagnosis involves serum ANCA testing and biopsy Diagnosis (remove all crusts and biopsy every turbinate) (remove Treatment is with steroids and cyclophosphamide with long Treatment term bactrim term Relapsing Polychondritis Relapsing Patients with this rare disorder produce antibodies Patients to type II collagen to Ear exam in acute case may reveal red, swollen Ear and tender ear with sparing of the lobule; over time, the ear becomes droopy time, Nasal chondritis may lead to saddle nose Nasal deformity deformity 50% of patients have laryngotracheal disease with 50% potential dynamic collapse of the trachea potential Sjogren’s Syndrome Sjogren’s Affects salivary and lacrimal glands May be primary (sicca syndrome) or secondary May (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 Autoimmune/Vasculitic Disorders Autoimmune/Vasculitic Wegener’s Granulomatosis Relapsing Polychondritis Sjogren’s Syndrome Bibliography 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 “Current Syndrome”. 100(6): 623-630 Syndrome”. Annals of Otology, Rhinology and Laryngology . “Lateral Laryngotomy for the Removal of Teflon “Lateral Granuloma”. 107:735-744 and “Rhinoscleroma Mimicking Nasal Polyposis” 110:290-292 Granuloma”. 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. Otolaryngologic Ferguson. 33(2) Apr 2002 Ferguson. Laryngoscope. “Necrotizing Sialometaplasia”. J. Gavron, J. Ardito, A. Curtis. July 1981 Vol 91 Laryngoscope. Kelley’s Textbook of Rheumatology 6th ed. Ruddy. 2001 Textbook of Primary Care Medicine 3rd ed. Noble. 2001 ...
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