Heavily testable facts Flashcards
1
Q
Chipmunk facies
A
Beta thalassemia
2
Q
Name the procedure for removing thyroglossal duct cyst
A
sistrunk procedure
3
Q
DX?
Syndromic association?
A
Hollman miller sign: Anterior bowing of the posterior wall of the maxillary sinus that occurs as a result of nasopharyngeal angiofibroma
Can be associated with Gardner’s syndrome
4
Q
Conditions that cause elevated vanillylmandelic acid (VMA) (3)
A
*note ALL neuroectodermally derived 1. Melanotic neuroectodermal tumor of infancy 2. Olfactory neuroblastoma 3. Pheochromocytoma
5
Q
Burton line
A
Bluish pigmentation of the marginal gingiva that results from lead poisoning
6
Q
3 Fun facts about chordomas
A
- most often seen involving the clivus
- contains physaliferous cells
- stains positive with brachyury
7
Q
Dermatofibroma staining vs dermatofibrosarcoma protuberans (DFSP)
A
DF: CD34- F13A+ DFSP: CD34+ F13A -
8
Q
Intestinal type sinonasal adenocarcinoma is associated with what professions
A
leather and woodworking
9
Q
What are 4 risk factors of sinonasal adenocarcinoma (not intestinal type)
A
- Southeast Asia
- EBV infection
- high salt fish diet (chinese male population greatly affected)
- vitamin C deficiency
10
Q
Warthin-Finkeldey giant cells are seen in what 5 conditions?
A
- measles
- lymphoma
- Kimura disease
- AIDs associated lymphoproliferative disorders
- Lupus erythematosus
11
Q
DX?
Name the two cell types indicated by the arrows
What is the causitive organism?
A
Rhinoscleroma caused by : Klebsiella rhinoscleromatis (gram negative bacteria)
Mikulicz cells(foamy histiocyte), Russell bodies (eosinophilic inclusions in plasma cells)
12
Q
Depicted entity:
- Chronic and localized infection of the mucus membranes
- Lesions present clinically as polypoid, soft masses (sometimes pedunculated) of the nose, throat, ear, and even the genitalia in both sexes.
What is the infectous organism?
A
Rhinosporiodosis- caused by Rhinosporidium seeberi (seems to be a fungus when you google it) -clinical presentation is an enlarged nostril/end of the nose
13
Q
Ascher syndrome features (3)
A
- Double lip 2. Goiter 3. upper eyelid edema (blepharochalasia)
14
Q
Paramedian lip pit associated syndromes (3)
A
- Van der woude syndrome 2. Kabuki syndrome 3. Popliteal pterygium syndrome
15
Q
Premature exfoliation of the mandibular incisors may be seen in
A
hypophosphatasia
16
Q
Beaten metal/copper skull is seen in which 3 conditions?
A
-Crouzan Syndrome (beaten-metal) -Apert syndrome (beaten metal) -Hypophosphatasia (beaten-copper)
17
Q
“Teeth floating in air” + skin plantar/palmar hyperkeratosis
A
Papilon-Lefevre syndrome
18
Q
Name that condition: Vertical bone trabeculae, Missing premolar, Retained primary molar
-List 2 other clinical findings
A
Segmental odontomaxillary dysplasia
Gingival overgrowth in the area of missing teeth
Becker nevus: hyperpigmentation with hypertrichosis
19
Q
Ghost teeth on radiology enameloid conglomerates on histo
A
regional odontodysplasia
20
Q
Black coated tongue cause
A
Bismuth staining
21
Q
Skull radiology: best diagnosis for punched out radiolucent lesions in a child vs an older adult
A
child: LCH adult: multiple myeloma
22
Q
A
Hydroxychloroquine pigmentation (antimalarial medication also given to lupus patients)
23
Q
Lateral Calcifications seen on PAN DDX (5)
A
- tonsilith
- sialolith
- phlebolith
- dystrophic acne
- calcified lymph node
24
Q
Globodontia (aka globe shaped teeth), hearing loss, and ocular colobomas are classic of what syndrome?
A
otodental syndrome
25
Papillary lesion of the soft palate DDX
1. squamous papilloma 2. verruciform xanthoma 3. Giant cell fibroma 4. Sialadenoma papilliferum
26
Munro's abscesses
psoriasis
27
Pautriers abscesses
Mycosis Fungoides
28
HLA-Cw6
psoriasis
29
Tissue culture pattern on histology
nodular fasciitis
30
DDX for mixed radiolucent-radiopaque lesion (6) (of course site and appearance play a factor)
CEOT
Ossifying fibroma
COC
odontoma
ameloblastic fibro-odontoma
AOT
31
Late stage florid COD- how do you rule out Pagets disease?
There should be no expansion in COD and ALK phos levels are normal (elevated in Pagets)
32
variant of ameloblastoma with mixed radiolucent/radiopaque appearance that resembles BFOL
desmoplastic ameloblastoma
33
A patient is diagnosed with basal cell carcinoma of the gingiva, what is the more likely diagnosis
a misdiagnosed ameloblastoma
34
-unerupted tooth, usually canine -radiolucency frequently extends below the CEJ of the tooth -snowflake calcifications
AOT
35
PTCH1 gene mutation occurs in what 2 entities?
OKC/Gorlin syndrome and CEOT
36
driven snow calcification appearance
CEOT
37
Amyloid-like acellular amorphous material occurring in CEOT is what?
odontogenic ameloblast-associated protein (ODAM)
38
Clear cell histology DDX (5)
1. Clear cell odontogenic carcinoma
2. hyalinizing clear cell carcinoma
3. Intraosseous mucoep
4. clear cell variant of CEOT
5. Metastatic dz: renal, breast, melanoma
39
A defect or groove in the palate may be caused by what odontogenic neoplasm
central odontogenic fibroma
40
Ground substance in an odontogenic myxoma is composed of
GAGs: predominantly hyaluronic acid and chondroitin sulfate
41
Clinical hallmarks: blue sclerae, hearing loss, joint hyperextensibility, contractures, triangular facies, frontal bossing, macrocephaly, flattened vertex and skull base, prominent occiput (back of the head)
3 conditions with blue sclera?
Osteogenesis imperfecta (brittle bone disease)
-Osteogenesis imperfecta, ehlers-danlos, marfan syndrome
42
4 Radiographic hallmarks of osteogenesis imperfecta
1. osteopenia
2. bowing of long bones
3. multiple fractures
4. increased number of wormian bones
43
Increased number of intrasutural bones (wormian bones) in what two conditions
-cleidocranial dysplasia -osteogenesis imperfecta
44
What disease: Spontaneous and progressive destruction of 1 or more bones (give the 5 names please)
What are the histo findings?
1.massive osteolysis, 2.vanishing bone disesae, 3.gorham disease, 4.gorham-stout disease, 5.phantom bone disease
Histo: Destroyed bone --\> vascular proliferation first → dense fibrous tissue
45
Many basophilic reversal lines within bone creating a characteristic jigsaw puzzle/mosaic appearance is characteristic histology for what?
paget's disease (osteitis deformans)
46
10 disorders with Multiple giant cell lesions (6 high yield)...What 2 entities have been associated with central giant cell granulomas in the jaw?
- **Cherubism**
- Noonan-like/multiple giant cell lesion syndrome
- **noonan syndrome**
- ramon syndrome
- **jaffe-campanacci syndrome**
**neurofibromatosis type 1**
- schimmelpenning syndrome
- oculo-ectodermal syndrome
**-pagets disease (giant cell tumors)**
**-hyperparathyroidism (brown tumors)**
JAW lesions assoc w central giant cell granulomas:
1. **Aneurysmal bone cyst**
2. **central odontogenic fibromas**
47
What cell lines are affected by early post zygotic activating mutation in GNAS
-Melanocytes, endocrine cells, osteoblasts \*if late in embryogenesis only osteoblasts are affected
48
Monostotic fibrous dysplasia that affects the maxilla and adjacent bones is termed
cranio-facial fibrous dysplasia
49
3 Syndromes associated with polyostotic fibrous dysplasia
1. Jaffe-lichtenstein
2. Mazabraud
3. McCune Albright
50
early presenting sign of polyostotic fibrous dysplasia is pain and long bone deformity- the deformity is specifically called what? (3 names)
deformity of the proximal femur = shepards crook deformity, coxa vara, hockey stick deformity (causes leg bowing)
51
Clinical: anterior mandibular COD with progressive growth
expansive osseous dysplasia
52
WHat diseasE?
- Diffuse fibro-osseous lesions of the jaws with prominent psammoma bodies
- bone fragility
- bowing/cortical sclerosis of the long bones
Gnatho-diaphyseal dysplasia GDD1 mutation
53
What disease?
- Parathyroid adenoma or carcinoma
- Ossifying fibromas of the jaws
- Renal cysts
- Wilms tumors
Hyperparathyroidism jaw tumor syndrome HRPT2 mutation
54
-Multifocal osteomas -Supernumerary teeth, impacted teeth, odontomas -adenomatous polyps
Gardner's syndrome APC gene chromo 5
55
Name 4 less frequently mentioned manifestations of Gardners syndrome
1. desmoid tumors- usually at the abdominal scar left after the patient receives colectomy
2. pigmented lesions of the ocular fundus: congenital hypertrophy of the retinal pigment epithelium
3. epidermoid cysts
4. thyroid carcinoma, pancreatic adeno, etc
56
-Radiology: radiolucent nidus with possible central opacity "target-like" -Clinical: nocturnal pain relieved by NSAIDs
osteoid osteoma (should also be less than 1.5-2cm in size)
57
Clinical: Genital lesions, conjunctivitis, arthritis, geographic tongue What condition? What HLA? Etiology?
-Reiter's syndrome (reactive arthritis) -HLA-B27 -occurs following venereal disease or dysentery
58
Multiple chondromas are associated with what 2 diseases/syndromes
1. Ollier disease: sporadic chondromatosis with unilateral tendency
2. Maffucci syndrome: sporadic chondromatosis and soft tissue angiomas
59
What syndromes increase the risk for osteosarcoma
Paget's disease
Li-fraumeni syndrome
Hereditary retinoblastoma
Rothmund-thomson syndrome
60
19 year old male
Bx: Non specific vascular proliferation with fibrous CT and chronic inflammatory cells
Massive osteolysis
Vanishing bone disease
phantom bone disease
gorham/gorham-stout disease
61
25 year old male
HX of veneral disease and arthritis
What is the term for associated genital lesions?
associated HLA?
Balanitis circinata
HLA-B27
62
5 year old female
Other findings: Bone surrounding the teeth is lower denisty, hyperplasia of the soft tissue in the area
DX?
Histo findings?
Regional odontodysplasia (ghost teeth)
Histo: enameloid conglomerates (also seen in amelogenesis imperfecta)
63
Clinical: 20 year old female; Tower skull(acrobrachycephaly) appearance
- Diagnosis?
- What is this image depicting?
- In more severe cases of this syndrome what type of skull deformity may occur?
-
- Apert syndrome
- Syndactyly
- Cloverleaf skull
64
55 year old male recently with new diagnosis and management of HTN
- DDX?
- What medications may cause this presentation?
- Medication related gingival hyperplasia vs Leukemic infiltrate
- (anti-epileptic)Phenyltoin, (immunosuppressant) cyclosporine, calcium channel blockers (amlodipine)
65
12 year old male
What is the most likely diagnosis?
What are these lesions?
List all of the manifestations of this disease
Mutation?
MEN2B
Mucosal neuromas
- Marfanoid body habitus, pheochromocytoma, medullary thyroid carcinoma
- 95% germline mutation at codon 918(M918T) of RET proto-oncogene (smaller subset have A883F)
66
- What is the term for the lesion shown?
- What % of patients with this lesion have an associated syndrome?
- What syndrome?
- What mutation and inheritance pattern?
- What other lesions would a patient with this syndrome have?
- Port wine stain (nevus flammeus)
- 8-10%
- Sturge Weber syndrome
- GNAQ chromo 9; not inherited
- Leptomeningeal angiomas, intellectual disability, contralateral hemiplegia, seizures
67
- Most likely diagnosis?
- What is the cause of the lesions?
- Associated genetics?
- Histologic buzzword?
- multifocal epithelial hyperplasia (Heck's disease)
- HPV 13 and 32
- HLA DR4
- Mitosoid cell
68
5 year old male with multiple rough itchy papules
- Diagnosis?
- Etiology?
- Patients with what 3 conditions are prone to prolonged or florid disease?
- Histo buzz words?
- Molluscum contagiosum
- poxvirus infection MCV
- HIV/AIDS, darier disease, atopic dermatitis
- Henderson paterson bodies
69
- What condition is the image depicting?
- Associated somatic mutation? (what other lesion has the same one?)
- If similar appearing lesions were throughout the body with associated pruritis what might you be concerned for?
- If the patient scratched one of these lesons before biopsy what is the charactertistic term for the histologic appearance?
- Dermatosis papulosa nigra
- FGFR3, PIK3CA (actinic lentigo)
- Leser-Trelat sign: rare phenomenon associated with internal malignancy
- Irritated sebK aka Inverted follicular keratosis of Helwig
70
Biopsy of one of these lesions shows sebaceous hyperplasia
- Name the associated syndrome
- Name 3 other findings in this syndrome
Muirre-Torre Syndrome
-visceral malignancies, sebaceous adenomas and carcinomas (shown in the image), keratoacanthomas
71
What is the most likely diagnosis?
- What other manifestations of disease would you expect?
- If the condition was limited to skin manifestations what is your DDX
- Noonan syndrome with multiple lentigenes (formerly LEOPARD syndrome)
- L:multiple lentigenes, E:electrocardiographic conduction defects, O: Ocular hypertelorism, P: pulmonary stenosis, A: abnormalities of the genitalia, R)etarded (slowed) growth resulting in short stature, D: deafness
- DDX: Lentiginosis profusa, Peutz Jeghers, LEOPARD
72
- The lesion seen is fully excised, what is the recurrence rate?
- what if it was verrucous?
- Transformation potential is this is mild vs moderate vs severe?
-10-35% for typical leukoplakia, 83% for verruciform variants
Mild: ~1-7%
Moderate: ~4-11%
Severe: 20-43%
73
This patient has been using smokeless tobacco for 10 years.
- What histologic features would be seen on biopsy?
- Clinical recommendations?
1. hyperkeratotic and acanthotic
- intraceullar vacuoles
- parakeratin chevrons
- amorphous subepithelial material in the superficial CT
\*dysplasia is uncommon
2. Habit cessation- lesion should go away in ~ 2 weeks, if persists 6 weeks+ then it should be biopsied
74
- Name 3 inherited syndromes associated with multiple of the lesion depicted in this image
- Name one acquired cause
Multiple keratoacanthomas:
**Inherited:**
Muir-Torre (sebaceous adenomas/carcinomas, KAs, GI carcinoma)
Ferguson-Smith syndrome (self-healing squamous epitheliomas, many KAs, Scottish descent)
Witten-Zak syndrome, multiple familial keratoacanthoma
**Acquired:**
Grybowski syndrome (hundreds/thousands small papules skin/GI-- may be associated with internal malignancy)
75
Multiple basal cell carcinomas are common in what inherited genetic disorders?
1. nevoid basal cell carcinoma syndrome
2. xeroderma pigmentosum
3. Albinism
4. Rombo syndrome
5. Bazex-Christol-Dupre syndrome
6. Dowling-Meara subtype of epidermolysis bullosa simplex
76
DX?
Laugier–Hunziker syndrome is a rare acquired disorder characterized by diffuse hyperpigmentation of the oral mucosa and longitudinal melanonychia in adults. They appear as macular lesions less than 5 mm in diameter.
77
Most commonly associated syndrome with this clinical presentation?
What other syndromes also have this lesion?
Port wine stain (nevus flammeus)
- Sturge Weber syndrome
- Beckwith-Weidemann syndrome
78
What is the term for this clinical presentation?
What are 5 causes?
1. Bell’s Palsy (idiopathic, viral, **Lyme disease**)
2. Ramsey Hunt Syndrome (**VZV** infection)
3. Heerdfort syndrome (**Acute sarcoidosis**: Uveitis, Parotid swelling, Fever, Facial paralysis)
4. Melkerson Rosenthal Syndrome (**Orofacial Granulomatosis**: lip swelling, facial nerve paralysis, fissured tongue)
5. Perineural/neural invasion of tumor affecting CN VII
79
Most likely diagnosis?
- Key radiographic features
- Key non-radiographic features
- What may have a similar presentation and confuse you
Segmental odontomaxillary dysplasia
- missing premolar, vertical oriented coarse bony trabeculae, smaller maxillary sinus
- Becker nevus, gingival hyperplasia in the area
- Fibrous dysplasia
80
All lesions with BRAF mutations
Some ameloblastomas
50% of Langerhan cell histiocytosis
Melanoma
Acquired melanocytic nevus
81
**The HLAs:**
- Cw6
- B27
- DR4
- DR3/B8
- DRw52
- B51
- DRB1
Cw6= psoriasis (geographic tongue)
B27= Reiters syndrome
DR4= Hecks disease
DR3/B8= primary sjogrens
DRw52=primary or secondary sjogrens
B51= Behcets
BRB1=Rheumatoid Arthritis
82
Mikulicz cells
Mikulicz disease
Mikulicz syndrome
Mikulicz aphthae
Mikulicz cells: rhinoscleroma (klebsiella rhinoscleromatis)
Mikulicz disease: IgG4 disease Bilateral painless swelling of lacrimal and salivary glands which show an intense chronic inflammatory infiltrate
Mikulicz syndrome: Similar parotid and lacrimal enlargement but due to TB, sarcoid, lymphoma etc
Mikulicz apthae: minor aphthous ulcers
83
Things to see with a wood's lamp
- Ash leaf spots of Tuberous sclerosis
- Red fluorescence of the teeth in Congenital Erythropoietic porphyria
- Give tetracycline 48 hours before a debridement for osteomyelitis: alive bone fluroescense with woods lamp and dead bone does not
84
_Some serologies:_
Diffuse cutaneous systemic sclerosis
Limited cutaneous systemic sclerosis (includes CREST)
Paget's disease
Lupus Erythematous
DCSS: antitopoisomerase-1 antibodies also called **anti-scl70**
LCSS: anticentromeric antibodies
Paget's: elevated ALK phos
Lupus: antibodies against dsDNA, antibodies against Sm(smith), elevated ANAs, elevated RF
85
DX?
Lichen planus
86
2 clinical syndromes of Acute Sarcoidosis
Lofgren syndrome:
Erythema nodosum
Bilateral hilar lymphadenopathy
Arthralgia
Heerfordt syndrome (uveoparotid fever)
Parotid enlargement
Anterior uveitis
Facial paralysis
Fever
87
What is the term for this histologic finding?
Diagnosis?
Asteroid body of sarcoidosis
-entrapped fragments of collagen within the granuloma
88
DX?
Name of the entity depicted?
Sarcoid granuloma
-Schumann body
89
What is the official / "more appropriate" name for the pattern seen on sialography of Sjogren's Syndrome?
punctate sialectasia (fruit-laden branchless tree)
90
Most likely diagnosis?
What is the etiology?
What is the clinical term applied to these lesions?
Bullous impetigo
Typically staph aureus only
Lacquer
91
What is the most likely diagnosis?
What are two other conditions might you consider in your DDX?
What is the clinical term sometimes used to describe the appearance of this entity?
Non bullous impetigo
DDX: HSV, exoliative chelitis(though not really in this picture)
Corn flakes glued to the surface of the lesion
92
What is the most likely diagnosis?
What is the etiology?
What two characteristic clinical terms are applied to this lesion?
Erysipelas
Group A beta hemolytic strep infection
peau d'orange & St. Anthony's fire
\*\*St anthonys fire is alos applied to ergotism aka ergot poisoning
93
This lesion is positive for staph aureus and strep pyogenes.
This is a rare clinical presentation of what disease?
What is the clinical term applied to this specific lesion?
What differentiates this particular clinical pattern from other clinical patterns of this same disease?
Impetigo
Ecthyma
The lesion heals with a permanent scar- scarring is not expected in other lesions of impetigo either bullous or nonbullous
94
List two conditions that can result in the lesion depicted
If this patient had chronic intraoral lesions what is the most likely diagnosis?
If this patient had chronic widespread involvement of oral, nasal, ocular, and laryngeal mucosa what autoantibody is likely?
If lesions associated with this disease process were isolated to the oral cavity what autoantibody is likely?
1. two conditions possible: Mucosal membrane pemphigoid (aka cicatricial pemphigoid) AND erythema multiforme
2. CHRONIC= Mucous membrane pemphigoid
3. widespread involvement by MMP is associated with autoantibody to epiligrin
4. Oral only involvement of MMP is associated with autoantibodies to alpha-6-integrin
95
Name that outdated and terrifying testing method:
Sterilized sarcoid tissue is injected into the skin of a patient
Kveim test
96
Olfactory neuroblastoma:
1. Name for low grade pseudo-rosette
2. Name for high grade true rosette
1. pseudo-rosette: Homer-Wright
2. true-rosette: Flexner-Wintersteiner
97
Diagnosis?
Congenital syphilis
98
Pupils that accomodate when patient looks at a close up object, but do not constrict with light are called _____ \_\_\_\_\_\_\_ pupils. This is seen in what condition?
Argyll Robertson pupils
Tertiary syphillis
99
Loss of coordination of movement in tertiary syphillis is termed?
tabes dorsalis
100
What is the diagnosis?
What other 2 clinical findings are part of the Hutchinson Triad?
Hutchinson teeth of Congenital syphilis
-Eigth nerve deafness, interstitial keratitis
101
BUGS summary:
## Footnote
Name the (most common) bug for each disease (keep scrolling):
- Diptheria
- Tuberculosis
- Syphilis
- Rhinoscleroma
- Rhinosporodosis
- Leprosy
- Gonorrhea
- Measles
- German measles
- Mumps
- Scarlet fever
- Impetigo
- Erysipelas
- Actinomycosis
- Cat scratch disease
- Histoplasmosis
- Blastomycosis
- Paracoccidomycosis
- Coccidioidomycosis
- Cryptococcosis
- Mucormycosis
- Toxoplasmosis
- Chicken pox
- Shingles
- Infectious mononucleosis
- NOMA
Diptheria = Corynebacteria Diphtheriae aka klebs loffler bacillus
Tuberculosis = Mycobacterium tuberculosis
Syphilis = Treponema Pallidum
Rhinoscleroma = Klebsiella rhinoscleromatis
Rhinosporodosis = Rhinosporidum seeberi
Leprosy = Mycobacterium leprae
Gonorrhea = Niesseria gonorrhoeae
Measles aka Rubeola = Paramyxoviridae, genus Morbillivirus.
German measles aka Rubella = Togavirus, genus Rubivirus
Mumps = Paramyxoviridae, genus Rubulavirus
Scarlet fever = Group A Beta hemolytic strep
Impetigo = staph aureus +/- strep pyogenes (aka group A, b-hemo)
Erysipelas = beta hemolytic strep (usually strep pryogenes)
Actinomycosis = actinomyces israelii
Cat scratch disease = Bartonella henselae
Histoplasmosis = Histoplasma capsulatum
Blastomycosis = Blastomyces dermatitidis
Paracoccidomycosis = Paracoccidioides brasiliensis
Coccidoidomycosis = coccidioides immitis &/or posadasii
Cryptococcosis = Cryptoccocus neoformans
Mucormycosis = Mucoromycotina absidia/mucor/rhizopus
Toxoplasmosis = toxoplasma gondii
Chicken pox = Varicella
Shingles = Herpes zoster
Infectious mononucleosis = epstein barr virus; HHV-4
NOMA = Fusobacterium necrophorum and Prevotella intermedia
102
This patient has anesthesia and loss of sweating of the overlying skin in these lesions. Biopsy demonstrated well formed granulomatous inflammation
Diagnosis?
Tuberculoid aka paucibacillary leprosy
103
Diagnosis?
What is the term for the characteristic facies?
What would you expect to see on biopsy?
Lepromatous (multibacillary) leprosy
- Leonine facies
- Sheets of lymphocytes with vacuolated histiocytes (aka lepra cells)
- No well formed granulomas
104
What is the general term for this clinical presentation?
What is the differential diagnosis?
Macrocheilia
105
Children with red teeth DDX
Congenital erythropoietic porphyria aka Gunther disease
Leprosy
106
What is the name for the cells depicted in this image?
What is the diagnosis?
Lepra cells of lepromatous leprosy- they are vacuolated macrophages
107
Other manifestations: fever, exanthematous rash, erythematous cheeks
What is the term for the clinical lesions depicted?
Diagnosis?
What oral lesions do you expect to see?
Pastia lines
Scarlet fever
-White strawberry tongue then red strawberry tongue
108
Bacterial species of chronic periodontitis red complex
- Treponema denticola
- Tannerella forsythensis
- Porphyromonas gingivalis
109
The area feels indurated or "wooden" to palpation
On incision of this lesion yellow granular material is seen.
DDX?
If histology shows club shaped filamentous bacteria in a radiating rosette pattern what is the diagnosis?
- Actinomycosis
- mimicked by Botryomycosis (usually caused by staph aureus)
- Definitive diagnosis is Actino
110
This patient also has hypoparathyroidism and hypoadrenocorticism.
Diagnosis?
Associated genetic mutation?
APECED: autoimmune polyendocrinpathy-candidasis-ectodermal dystrophy syndrome
- mucocutaneous candidiasis, nail dystrophy, corneal keratopathy, hypoparathyroid/hypoparathyroid
- childhood onset
- Mutation in AIRE gene
111
These are two different presentations of the same disease.
What is the terminology used to describe each image?
What is the diagnosis?
Luetic glossitis = atrophic form
Interstitial glossitis = gummatous form
Tertiary syphilis
112
Patient has a fetid odor eminating from their mouth. Past medical history is signficant for smoking. What is the best diagnosis?
- What bug is implicated in this process?
- Risk factors associated with this process?
- If there was no associated odor what else might you consider on the differential?
- If this disease process spreads from the gingiva to the adjacent soft tissue what term would then apply?
- If this disease process becomes chronic and causes the loss of alveolar bone what term would apply?
- If this disease spread through multiple planes of soft tissue and onto the skin of the face what term would then apply?
**Necrotizing ulcerative gingivitis**
- Fusobacterium nucleatum, prevotella intermedia, porphyromonas gingivalis, treponema spp., Selenomonas spp.
- Smoking, Stress, Poor nutrition, AIDS, immunosuppresion, Poor nutrition
- Neisseria gonorrhea infection
- Necrotizing ulcerative mucositis
- Necrotizing ulcerative periodontitis
- Cancrum oris (NOMA)
113
PMH: Healthy 35 year old male, areas have been present since adolescence. Most likely diagnosis?
- In a 55 year old male with a clinical history significant for heart transplant what diagnosis is more likely?
- Name 3 associated familial syndromes
- TX?
**-Gingival Fibromatosis**
## Footnote
- Medication induced gingival hyperplasia probably 2/2 cyclosporine and/or CCB
- Byars-Jurkiewicz syndrome, Costello syndrome, Cross syndrome, Infantile systemic hyalinosis, Jones-Hartsfield syndrome, Ramon syndrome and more
- Excellent oral hygiene +/- surgical revision based on severity (better outcome when you wait until after all permnanent teeth erupted)
114
High yield genetic disorders associated with periodontitis in kids/young adults
What is the specific terminology applied to this?
**Papilon Lefevre & Haim munk**
**Acrodynia (pink disease of mercury poisoning)**
**Ehlers Danlos**
**Hypophosphatasia**
**Trisomy 21**
Others: Acatalasia, Chediak Higashi syndrome, Cohen syndrome, AIDS, DM, Kindler syndrome
-Periodontal disease as a manifestation of systemic disease
115
What is the predominant bacteria of localized aggressive periodontitis?
This bacteria has also been found prevalently in the oral disease of patients with what disease?
**-A. Actinomycetemcomitans (**Aggregatibacter actinomycetemcomitans)
**-Papilon Lefevre**
Bacteria types expand when patients have generalized type and include the red complex
116
What is the term for the histologic feature seen in this image?
What conditions/diseases can this be found in?
Hamazaki-Wesenberg Bodies aka yellow-brown bodies
myriad medical conditions: appendicitis, cirrhosis, lymphoid tumours, colon carcinoma and numerous others, most famously sarcoidosis
117
**Viral/Fungal/Bacterial Buzz words, name that disease:**
- Koplik spots
- Mickey mouse ears/Mariners wheel histology
- Forchheimer sign
- Signficant cause of death in AIDS pts
- Doubly refractile cell wall
- Nine banded armadillo carries this
- How about a regular armadillo carrier
- Frequent overlying pseudoepitheliomatous hyperplasia on bx
- Female hormones are protective against?
- Hypersensitivity reaction to this is termed valley fever
- Cutaneous form of disease has lesions that resemble a volcano
- Mulberry-like ulcerations
- Prominent mucopolysaccharide capsule (stain w/mucicarmine)
- Pts taking deferoxamine or who are ketoacidotic at higher risk
- Fruiting bodies
- Obligate intracellular parasite
- Black fever aka kala-azar
_Koplik spots_ = Measles (Rubeola) (Paramyxoviridae, Morbillivirus)
_Mickey/Mariner_ = Paracoccidioidomycosis (Paracoccidioides brasiliensis)
_Forchheimer sign_ = German Measles (Rubella) (Toga virus Rubivirus)
_Signif COD in AIDs pts_ = cryptococcosis (Cryptococcus neoformans)
_Doubly refractile cell wall_ = Blastomycosis (Blastomyces dermatitidis)
_Nine banded armadillo_ = Paracoccidioidomycosis (Paracoccidioides brasiliensis)
_Regular armadillo_ = Leprosy (Mycobacterium leprae) (Hansen disease)
_Freq pseudoepi hyper_ = Blastomycosis (Blastomyces dermatitidis)
_Female hormones protective_ = Paracoccidioidomycosis (Paracoccidioides brasiliensis)
_Hypersensitivity rxn to coccidioidomycosis_ = valley fever (Coccidioides immitis or posadasii)
_Cutaneous volcano_ = Leishmaniasis (species Leishmania)
_Mulberry ulcerations_ = Paracoccidioidomycosis (Paracoccidioides brasiliensis)
_Prom mucopolysaccharide capsule_= cryptococcosis (Cryptococcus neoformans)
_Increased iron_ = increased risk of mucormycosis (Mucormycotina Rhizopus)
_Fruiting bodies_ = aspergillosis (Aspergillus fumigatus)
_Obligate intracellular parasite_ = leishmaniasis (species Leishmania)
_Black fever_ = visceral leishmaniasis (species Leishmania)
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Direct immunofluorescence pattern for Chronic ulcerative stomatitis?
Indirect immunofluorescence finding?
- speckled pattern of IgG deposits in the basal one-third of the epithelium
- Indirect: presence of stratified epithelium specific anti-nuclear antigen (SES-ANA)
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What is the characteristic cell depicted here?
What is this diagnostic of?
Floret cell
Pleomorphic lipoma
120
In addition to the clinical finding below this patient is also experiencing joint pain.
What is the most likely diagnosis?
List the features of this condition
SAPHO syndrome
Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis
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Multiple bone involvement by primary chronic osteomyelitis in children is termed
CRMO: Chronic recurrent multifocal osteomyelitis
thought to be a widespread variant of primary chronic osteomyelitis
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This finding is present on both the hands and feet in addition to a pruritic skin rash.
DDX?
- If this was a 5 year old patient with red eyes and cracked lips in addition to these findings, what diagnosis would you favor?
- If this patient had HTN and neurologic symptoms what diagnosis would you favor? What is the term used for the neurologic symptoms?
\*What oral manifestations would you expect in the second case?
- Acrodynia, Kawasaki disease, Polycythemia Vera (erythromelalgia)
- Kawaski disease
- **Acrodynia**; neurologic symptoms are termed Erethism-excitability, tremors, memory loss, delirium
- Oral symptoms of acrodynia: excessive salivation (also excessive sweating) and premature tooth loss
-
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34 year old male, HIV+, lesions are also present on the palms and soles of the feet
DDX for this case?
-What conditions cause a rash that includes the palms and soles of the feet?
Secondary syphilis
Psoriasis (specifically guttate)
Erythema multiforme (should expect more oral manifestations not shown)
Palms and soles of the feet inclusive rashes: syphilis, psoriasis, EM, coxsackie viruses, rocky mountain spotted fever, mycosis fungoides, tinea
124
DX?
Calcific metamorphosis and aseptic pulpal necrosis
125
What are the two major clinical findings?
What two conditions produce these radiographic findings?
- Thistle tube/flame shaped pulp chambers & pulp stones
- Dentin dysplasia type 2 and pulpal dysplasia
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What termed is applied to this radiographic finding?
What is the diagnosis?
What is the histologic description of the root of these teeth?
Rootless teeth
Dentin dysplasia type 1
Histo buzz:
- whorls of tubular dentin
- “stream flowing around boulders”
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What two pathoses can cause a "bull neck" appearance
Diptheria
Ludwigs angina
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**Whats my keratin?**
Epidermolysis Bullosa simplex
White sponge nevus
Pachyonychia congenita
Oral lesions of pachyonychia congenita
Pachyonychia congenita with neonatal teeth
EBS: Keratins 5 & 14
WSN: Keratin 4 & 13
Pachyonychia congenita: 6a, 6b, 16, 17
Pachyonychia with oral lesions: Keratin 6a
PC neonatal teeth: Keratin 17
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At least 4 conditions associated with premature tooth loss
Acrodynia (pink disease) mercury exposure children
Hypophosphatasia
Dentin Dysplasia type 1 (rootless teeth)
Papilon Lefevre
Haim Munk
Acatalasia
Cohen Syndrome
Ehlers-Danlos
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What clinical features are depicted in this image?
What is the most likely diagnosis?
What mutation and inheritance pattern is associated with this condition?
-bowing of long bones with a break (fragility of the bones), diffuse fibro-osseous lesions of the jaws (on histology they have prominent psamomma bodies)
MUT: GDD1, auto dom
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Time to dominate Lipid Reticuloendothelioses....
What are the three diseases & their subtypes?
1. Goucher (Type 1 - least severe, Ashkenazi Jewish, Types 2/3 CNS invovment, severe)
2. Neimann Pick (ABC: A,C=severe, B=less severe)
3. Tay-Sach (Mild to severe: mild can live to adulthood, severe do not)
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What is the image depicting?
What disease is this associated with?
What is another important maxillofacial feature?
What is the primary histological buzz phrase?
What enzyme is lacking? What accumulates?
Erlymeyer Flask Deformity (EFD) of the longbones
Gaucher Disease
Multiple RLs of the mandible (filled with Gaucher cells)
Gaucher cells (macrophages) with wrinkled silk cytoplasm
Glucocerebrosidase missing so accumulation of glucosylceramide
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Niemann Pick Disease:
What enzyme is missing? What accumulates as a result?
Of the types, which are more severe?
What is the genetic mutation?
What is the characteristic cell?
Sphingomyelinase missing, so sphingomyelin accumulates
A,C are most severe...Type B survives into adulthood
NPC1, NPC2
"Sea-Blue" Histiocyte seen on bone marrow aspirate
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Tay-Sachs:
How does it compare to the other lipoidendoreticuloses?
What enzyme is missing? What accumulates?
What cell type is affected?
Most severe of the lipoidendoreticuloses...only mild cases survive to adult hood
Beta-hexosaminidase A missing, ganglioside accumulates
Neurons (hense the severe nature as opposed to Gaucher and NP where accumulation happens in macrophages)
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Infant with bilateral facial swellings, fever, leukocytosis, and hyperostosis of his facial bones. Blood cultures negatve. What's the most probable diagnosis?
Caffey Disease (AD, a COL1A1 mutation has been found)
136
Name the skin lesion...What systemic condition is it associated with?
Lupus pernio. Sarcoidosis
Lupus pernio is a chronic raised indurated (hardened) lesion of the skin, often purplish in color. It is seen on the nose, ears, cheeks, lips, and forehead. It is pathognomonic of sarcoidosis.
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What are the 3 main components of RAMON syndrome?
1. Cherubism
2. Gingival fibromatosis
3. seizures
138
What is the most likely diagnosis for for lesion depicted?
What syndrome is associated with these lesions?
What are the major components of this syndrome?
- Multiple large congenital nevi
- Neurocutaneous melanosis
- Potentially fatal condition in which patients have multiple or large congenital nevi + melanocytic neoplasms of the CNS (melanomas)
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What is the term for areas indicated by the yellow arrows?
What diagnosis is this feature specific for?
What mutation is associated?
- Kamino bodies
- Spitz nevus
- HRAS
140
- What is the most likely diagnosis?
- What is the most common mutation associated with this lesion overall?
- What is a common mutation associated with this lesion at this specific site?
- What are the 4 clinicopathologic subtypes of this entity?
which subtype is most common in the oral cavity?
which subtype is most common in Blacks?
which subtype is most common on the skin?
- Melanoma
- BRAF 50%
- Mucosal melanomas are also associated with KIT mutations
- Clincopathologic subtypes:
superficial spreading, nodular, acral lentigenous, lentigo maligna melanoma (lentigo maligna aka hutchinson freckle is the precursor lesion and is just melanoma in situ)
- Oral cavity = acral lentigenous
- Blacks = acral lentigenous
- Skin = superficial spreading (70%)
141
A 75 year old male presents with complaints of right sided jaw and neck pain. IOE is WNL. EOE is positive for the image shown.
- What is the most likely diagnosis?
- What would you see on biopsy?
- What other symptoms are classic for this condition?
- Temporal arteritis aka giant cell arteritis aka Horton disease
- transmural and perivascular infiltrate of lymphocytes, histiocytes and giant cells +/- granulomas. Elastic layer is fragmented
- blurry vision, headaches, jaw pain
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This biopsy is from the posterior lateral tongue.
Diagnosis?
- is this a neoplastic process?
- Sub-gemmal neurogenous plaque
- This is part of normal anatomy. Plexus underlies taste buds on the posterior lateral tongue. Taste buds are CK 8/18+
143
This is a biopsy of an enlarged cervical lymph node.
On higher power the following are seen: Karryorhexis, fibrin deposits, plasmacytoid monocytes
- Most likely diagnosis?
- What other entities are considered on the differential? How would you exlcude them?
- Kikuchi disease
- Cat scratch disease: there should be a history of cat exposure and a skin papule. You also typically see follicular hyperplasia and more neutrophils
- Lymphoma with necrosis: should see more atypia and no plasmacytoid monocytes
- Lupus lymphadenitis: architecture of the lymph node is presevered, necrosis is in the paracortical area
144
Patient presented with a well-defined, oval, red nodule of the skin.
Diagnosis?
- Is this process benign, malignant, or reactive/nonneoplastic?
- What are the 3 histologic components of this entity?
Intravascular papillary endothelial hyperplasia (Massons tumor)
- Non neoplastic/reactive process
- Dilated vascular spaces, fibrin deposition, endothelial cell proliferation
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6 year old patient, Diagnosis?
- Characteristic finding on electron microscopy?
- stains?
What classification system is currently used?
- LCH
- Birbeck granules
- CD1a, langerin (CD207)
CLASSIFICATION:
1. Single organ: usually bone or skin, can be multiple lesions but only of one system
2. Multiorgan w/o organ dysfunction
3. Multiorgan w/ organ dysfuction low risk: skin, LN, pituitary, bone
4. Multiorgan w/ organ dysfunction high risk: lung, liver, spleen, BM
146
This clinical presentation is characteristic for what?
What is the responsible genetic mutation?
The mutation results in overproduction of what product?
What is the inheritance pattern?
What 2 other conditions/diseases can produce a similar appearance of the body habitus?
- Marfan syndrome
- Fibrillin-1
- TGF-Beta
- Autosomal dominant
- MEN2B, NBCCS
147
A 20 year old patient present with a well-defined radiolucency of the mandible. All teeth in the area are vital. A biopsy is performed and shows the following.
- What is the histologic buzzword for this presentation?
- What is this characteristic (but not specific) for?
- What stains would you perform for confirmation?
- What is the classic clinical presentation for this disease?
- What is the prognosis/outcome for the majority of patients?
- Emperipolesis: the active penetration of one cell by another which remains intact
- Rosai-Dorfman disease aka sinus histiocytosis with massive LAD
- S100 and CD1a: S100 positive (same as langerhan cell histiocytosis), CD1a negative (to rule out langerhan histiocytes)
- Massive painless cervical LAD in a younger patient, of unknown etiology
- 80% of cases spontaneous resolve, this is not a clonal process
148
149
Diagnosis?
Term for the foot manifestation?
Oral findings?
- Arsenic poisoning
- Black foot disease
- necrotizing ulcerative stomatitis, excessive salivation,
150
This patient was previously treated with gold.
-What is the term for manifestation seen in this photo and what is insighting event?
What is a common oral complication in patients treated with gold?
Chrysiasis: when patients are treated with gold therapy they can develop this slate blue pigmentation in areas exposed to sunlight
-Oral mucositisi
151
What bacteria is most commonly responsible for Lemierre syndrome?
Fusobacterium necrophorum
- usally occurs in teens
- can be life threatening
- basically an oropharyngeal infection that can lead to sepsis
152
A newborn is born with jaundice. If the baby is not treated these may develop ______ a dangerous accumulation of bilirubin in the brain
Kernicterus
153
DDX?
- This patient had peripheral blood eosinophilia, what is the diagnosis?
- A biopsy is performed of the lymph node, what would you expect to see?
- DDX: Branchial cleft cyst, Unicentric castleman disease, kikuchi disease, kimura disease, metastatic (HPV+ SCC) carcinoma
- Kimura disease
- eosinophils, eosinophilic microabscesses, vessels with hyalinzation, follicular hyperplasia
154
Diagnosis?
-Cholesteatoma
155
A patient complains of profuse sweating near her right ear when eating spicy and sour foods. PMH signficant for previous excision of a pleomorphic adenoma in the parotid glad. WHat is the most likely diagnosis? What test could be used to better demonstrate the patients symptoms?
- Frey's syndrome: results from injury of the salivary gland
- Starch iodine test
156
Local causes of delayed tooth eruption
Systemic causes of delayed tooth eruption
Local causes: gingival fibromatosis, clefts, ROD, SOD, supernumeraries, tumors
Systemic: anemia, celiac, cerebral palsy, chemotherapy, endocrine disturbances, vit D resistant rickets
157
DX?
What intraoral finding is found in 92% of these patients
Rubinstein-Taybi
Talon cusp
158
Dens en dente aka dens invaginatus
159
DX?
What might this be caused by?
buccal bifurcation cyst
-cervical enamel extension
160
What syndrome would you suspect?
What are the other associated findings?
PHACES
P: posterior fossa brain anomalies
H: Hemangioma
A: arterial anomalies
C: Cardic defects
E: Eye anomalies
S: sternal cleft, supraumbilical raphe
161
What is the term for this finding?
Name an associated syndrome?
sternal cleft
PHACES
162
What is the term for this radiographic finding?
What is it characteristic of?
tram-track or tram-line calcifications
Sturge weber syndrome
163
Salt fish diet --\>
Leather and wood-working --\>
salt fish = sinonasal adenocarcinoma
leather and wood-working = intestinal type sinonasal adenocarcinoma
164
The child has had multiple episodes of swelling
There is no pus on palpation of the duct
DX?
TX?
Is this condition the most common cause worldwide of parotid swelling?
Juvenile recurrent parotitis
Usually happens around 3-6 yrs old, multiple episodes, seld resolves by puberty
-Worldwide: Mumps, in the USA: Juvenile recurrent parotitis
165
DX?
Cheilitis glandularis
166
What conditions can produce complaints of excess drooling?
If there is no obvious cause, and they get episodic sialorrhea, what is the term?
- cerebral palsy, down syndrome, ALS, parkinsons, heavy metal poisoning, rabies
- idiopathic paroxysmal sialorrhea
167
Rose bengal dye test is used for?
What other test is used for the same purpose?
- diagnosis of keratoconjunctivitis sicca
- Schirmer test
168
Difference between Epstein Pearl vs Bohn Nodule vs Ging Cyst of Newborn
Epstein pearls: traped epithelium along fusion MIDLINE
Bohns Nodule: minor salivary glands, scattered over hard palate, near soft palate junction
Gingival cyst of newborn: dental lamina, on alveolar ridge
169
Conditions that affect the nails (8)
Lichen planus: thinned with longitudinal ridging and fissuring
Hand foot mouth disease: Beau lines, Onychomadesis(spontaneous separation of nail from matrix)
Plummer-vinson: Koilonychia aka spoon shaped nails
APECED/mucocutaneous candidiasis
Pachyonychia congenita: Nail thickening
Dyskeratosis congenita: nail dystrophy
Dariers disease: candy-cane nails (alternating red and white bands)
Laugier-Hunziker: longitudinal pigmented band on the nail
170
Large radiolucency is seen in a skull film of a patient with elevated alk phos and bone pain. What is the clinical term for this lesion?
osteoporosis circumscripta- occurs in early stage disease
171
What 3 disease cause Forcheimer spots
German measles (rubella)
Scarlet fever
Mono
172
Lesion is firm
BX demonstrates benign appearing bone and cartilage
Cutright lesion AKA Reactive osseous and chondromatous metaplasia
173
Diagnosis?
Recommended therapy?
DX: Perioral dermatitis
1. "zero therapy" tell the patient to discontinue anything they have been applying in the area
2. topical pimecrolimus or topical erythromycin.
3. Oral tetracycline (though not in children or pregnant patients)
174
DX?
Tx?
DX: Exfoliative chelitis
TX:
1. Elimination of habit
2. Tx of any superimposed infection: antiobiotic and or antifungal
3. topical tacrolimus
175
DX?
TX?
DX: transient ligual papillitis
TX: self resolving though you can give topical corticosteroids or analgesics for discomfort
176
Differential DX?
Ancillary testing to confirm dx?
Behcet's syndrome, IgA deficiency, cyclic neutropenia, IBD, Sweet syndrome, MAGIC syndrome
Ancillary test for Behcets = positive pathergy test
cyclic neutropenia = concurrent blood tests, biopsy that shows little neutrophil acitivity in the ulcer
177
Describe the finding being depicted in this image. What systemic condition(s) is this typical of?
Loss of lamina dura- generalized
- Hyperparathyroidism, osteomalacia, pagets disease, fibrous dysplasia
178
In addition to the finding depicted this male child has the following bloodwork:
## Footnote
- Hypophosphatemia
- Low serum calcium
What is the most likely diagnosis?
What characteristic finding is seen on histology of one of these teeth?
The picture is showing an abscess which frequently results from pulpal extensions to the surface in Vitamin D resistent rickets
\*\*teeth on histology demonstrate globular dentin
179
What is this image depicting and where is this commonly found?
Hyaline body aka pulse granuloma
Walls of inflammatory cysts
