General oto manifestations of systemic disease Flashcards
What is eosinophilic granuloma?
Eosinophilic granuloma is the most common form of
Langerhans cell histiocytosis and is characterized by the
formation of solitary or multiple discrete nodules within
bones.
What is Hand-Schüller-Christian disease?
It is multifocal Langerhans cell histiocytosis with bone
“granulomas,” associated with the triad of exophthalmos,
lytic skull lesions, and diabetes inspidus.
True or false. Most patients with Langerhans cell
histiocytosis have multisystem disease.
False. Approximately two-thirds of patients have disease
involving one site.
What is Letterer-Siwe disease?
Systemic Langerhans cell histiocytosis is the rarest (~ 10%
of cases) and the most severe form of disease. Initial
symptoms often include generalized skin eruption, anemia,
and hepatosplenomegaly.
Extranodal natural killer cell (NK)/T-cell lymphoma,
nasal type, is commonly associated with which
virus?
Epstein-Barr virus
How is extranodal NK/T-cell lymphoma, nasal-
type, commonly treated?
Chemotherapy (CHOP: cyclophosphamide, doxorubicin,
vincristine, prednisone) in conjunction with radiotherapy
Compared with other types of lymphoma involv-
ing the head and neck, what is the prognosis for extranodal NK/T-cell lymphoma, nasal type?
Much poorer
What is the most common laryngeal manifestation
of Wegener granulomatosis?
Subglottic stenosis
What is the most common otologic manifestation
of Wegener granulomatosis?
Serous otitis media
What histopathologic findings are seen in Wege-
ner disease?
Necrotizing granulomas and arteritis involving small vessels
What laboratory test is used to diagnose and
follow Wegener disease?
Antineutrophil cytoplasmic antibody (C-ANCA). Test will be
positive in more than 90% of cases.
What histopathological findings are seen in
sarcoidosis?
Noncaseating granulomas
What condition is characterized by uveitis, parotid
swelling, fever, and facial nerve palsy?
Heerfordt syndrome (uveoparotid fever), a manifestation of sarcoidosis
What is the most common site of laryngeal
involvement of sarcoidosis?
Supraglottis
What is the most common head and neck mani-
festation of sarcoidosis?
Cervical lymphadenopathy
Describe a common presentation for pyogenic
granuloma.
Less than 1-cm lobular red or purple lesion that develops on
the gingiva or nasal mucosa that is friable and prone to
bleeding. It occurs more commonly in women and is
associated with trauma and pregnancy.
Describe the treatment of pyogenic granuloma.
Lesions frequently resolve spontaneously when associated
with pregnancy. If frequent bleeding or cosmesis is a concern,
conservative cauterization or excision can be pursued.
Describe the natural history of untreated necro-
tizing sialometaplasia.
Spontaneous resolution over weeks to months
How does blastomycosis typically manifest?
A common triad includes cutaneous disease, pulmonary
involvement, and constitutional symptoms. Skin lesions
may involve the face with verrucous growth and scarring.
Oropharyngeal and laryngeal involvement are rare.
Describe the clinical manifestations of coccidiomycosis.
It is the cause of “valley fever.” Most patients have an
influenza-like illness that includes malaise, fever, myalgia,
arthralgia, and cough. Initial symptoms are pulmonary,
mucocutaneous involvement with maculopapular rash,
cervical lymphadenopathy, and/or meningeal involvement.
Where is Coccidioides immitis endemic?
Desert Southwest including New Mexico, Nevada, Califor-
nia, Texas, Utah, and northern Mexico
Histoplasmosis most commonly occurs in which
geographic location?
The Ohio, Missouri, and Mississippi River valleys
Describe the head and neck manifestations of
disseminated histoplasmosis.
Granulomatous lesions involving the lips, gingiva, tongue,
larynx, and pharynx manifesting with painful ulcers
containing heaped edges
What is the most common site of laryngeal
involvement of histoplasmosis?
Supraglottis
How is histoplasmosis diagnosed?
Histoplasma antibody latex agglutination test, fungal stains,
blood or urine antigens using enzyme-linked immuno-
sorbent assay or polymerase chain reaction (PCR), or
histoplasma skin test
What pathogen is responsible for the development
of rhinosporidiosis?
The parasite Rhinosporidium seeberi
What are the common head and neck clinical
manifestations of rhinosporidiosis?
Fleshy, friable strawberry-like lesions most commonly
involving the inferior turbinate, oropharynx, conjunctiva,
and perineum
What is the treatment for rhinosporidiosis?
Wide local excision or prolonged dapsone therapy
What is the causative organism in syphilis?
The spirochete Treponema pallidum
What is the treatment of syphilis?
Penicillin G benzathine
Describe the presentation of primary syphilis.
A painless ulcer (chancre) at the site of transmission
demonstrating a rolled edge and punched-out base is
present after 3 to 6 weeks at the site of exposure.
Describe the presentation of secondary syphilis.
Secondary syphilis is characterized by systemic spread of
disease with manifestations including fever, myalgias,
arthralgias, and lymphadenopathy. A mucocutaneous rash
often develops including the oral mucosa and the palms
and soles. Finally, condyloma lata and patchy alopecia may
develop.
What are the three categories of tertiary syphilis?
The three categories are gummatous syphilis, cardiovascu-
lar syphilis, and neurosyphilis.
Describe the findings of congenital syphilis.
“Snuffles” with sinonasal drainage, saddle nose, saber shins,
Hutchinson teeth, and mulberry molars
Describe the Argyll-Robertson pupil.
A pupil that does not react to light but does constrict
during accommodation. Associated with syphilis
Describe the manifestation of otosyphillis.
Otosyphilis is associated with either congenital or tertiary
acquired syphilis and manifests with sensorineural hearing
loss beginning in the high frequencies, fluctuating tinnitus,
and vertigo.
What is the Hennebert sign?
Rotary nystagmus when positive or negative pressure is applied to the tympanic membrane
Describe the Jarisch-Herxheimer reaction.
After treatment of syphilis, dying spirochetes may trigger a
cytokine cascade that manifests with myalgias, fever,
headache, and tachycardia.
What tests are commonly used for syphilis
screening?
Venereal Disease Research Laboratory (VDRL), rapid plasma
reagin (RPR) test
What confirmatory test should be ordered after a
positive or equivocal screening test for syphilis?
FTA-ABS
What is the most common cause of subacute
pediatric cervical lymphadenopathy?
Atypical Mycobacterium, most commonly M. avium-intra-
cellulare, M. haemophilum, and M. scrofulaceum
How is brucellosis transmitted?
It is transmitted from contaminated meat or dairy products
or via direct contact through broken skin.
What are the clinical manifestations of cat-scratch
disease?
The primary lesion develops as an erythematous, non-
pruritic pustule 1 week affter inoculation. Lymphadenitis of
the axilla, neck, and inguinal region commonly develops 2
to 4 weeks after exposure. Suppuration with acute tender-
ness and fever frequently occurs. Lymphadenopathy usually resolves over 2 weeks but may persist for up to 2 years.
What are the pathogens associated with leprosy
(Hansen disease)?
Mycobacterium leprae and Mycobacterium lepromatosis
What is the treatment for Hansen disease?`
Dapsone
What pathogen is responsible for the development
of rhinoscleroma?
Klebsiella rhinoscleromatis
What histologic findings are strongly suggestive of
the diagnosis of rhinoscleroma?
Russell bodies (immunoglobulin containing inclusions in plasma cells), pseudoepitheliomatous hyperplasia, Mikulicz cells (foamy histiocytes containing Klebsiella)
What is the treatment for rhinoscleroma?
Tetracycline or ciprofloxacin
What tests are helpful in diagnosing systemic
lupus erythematosus?
Antinuclear antibody (ANA), anti-Sm, anti-DNA, anti-ribo- nuclear protein (anti-RNP), and anticardiolipin antibody
What are the typical clinical manifestations of a
patient with mixed connective tissue disease?
Raynaud phenomenon, arthralgias, inflammatory myopa-
thy, lymphadenitis, skin or mucosal lesions, and serositis
What is the definition of mixed connective tissue
disease?
Mixed connective tissue disease is characterized by a
combination of overlapping features of systemic lupus
erythematous, scleroderma, and polymyositis. Patients may
manifest Raynaud phenomenon, arthralgias, inflammatory
myopathy, lymphadenitis, skin or mucosal lesions, and
serositis.
Which joints in the head and neck are most
commonly affected by rheumatoid arthritis (RA)?
RA can affect any diarthrodial joint, including the tempor-
omandibular, cricoarytenoid, and ossicular joints.
What does the acronym CREST stand for?
Calcinosis, Raynaud phenomenon, esophageal dysmotility,
sclerodactyly, and telangiectasias. CREST syndrome is a
limited cutaneous form of systemic scleroderma.
What are the histologic findings in a minor salivary
gland lip biopsy performed in a patient with
Sjogren disease?
Focal lymphocytic infiltrate with atrophic acini
What head and neck sites are commonly affected
by relapsing polychondritis?
Ear, nasal septum, and larynx
What is the most common head and neck mani-
festation of relapsing polychondritis?
Episodic auricular chondritis, presenting with erythema and
pain of the pinna with sparing of the fatty lobule. Eventually
presents in ~ 90% of patients
What are common symptoms of giant cell
arteritis?
Headache (predominantly temporal region), jaw claudica-
tion (~ 50% patients), vision loss or visual disturbance,
fatigue, general malaise, fever, anorexia, weight loss, night
sweats, and tongue pain
What histologic finding on temporal artery biopsy
is suspicious for giant cell arteritis?
Inflammatory infiltrates in at least the adventitia and media,
with elastic lamina fragmentation
What imaging study may be useful in the diagnosis
of giant cell arteritis?
Duplex ultrasound. High-resolution MRI has been reported
to have very similar diagnostic power, but more data are
needed before it can be recommended as a diagnostic tool.
What is the classic triad of Wegener granuloma-
tosis?
Granulomas of the respiratory tract, progressive glomer-
ulonephritis, and necrotizing vasculitis of small to medium-
sized arteries and veins
What condition is characterized by uveitis, oral
aphthous ulcers, and genital ulcers?
Behçet disease
