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Fellowship and boards study > Heavily testable facts > Flashcards

Heavily testable facts Flashcards

1
Q

Chipmunk facies

A

Beta thalassemia

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2
Q

Name the procedure for removing thyroglossal duct cyst

A

sistrunk procedure

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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

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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

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5
Q

Burton line

A

Bluish pigmentation of the marginal gingiva that results from lead poisoning

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6
Q

3 Fun facts about chordomas

A
  1. most often seen involving the clivus
  2. contains physaliferous cells
  3. stains positive with brachyury
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7
Q

Dermatofibroma staining vs dermatofibrosarcoma protuberans (DFSP)

A

DF: CD34- F13A+ DFSP: CD34+ F13A -

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8
Q

Intestinal type sinonasal adenocarcinoma is associated with what professions

A

leather and woodworking

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9
Q

What are 4 risk factors of sinonasal adenocarcinoma (not intestinal type)

A
  1. Southeast Asia
  2. EBV infection
  3. high salt fish diet (chinese male population greatly affected)
  4. vitamin C deficiency
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10
Q

Warthin-Finkeldey giant cells are seen in what 5 conditions?

A
  1. measles
  2. lymphoma
  3. Kimura disease
  4. AIDs associated lymphoproliferative disorders
  5. Lupus erythematosus
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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)

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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

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13
Q

Ascher syndrome features (3)

A
  1. Double lip 2. Goiter 3. upper eyelid edema (blepharochalasia)
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14
Q

Paramedian lip pit associated syndromes (3)

A
  1. Van der woude syndrome 2. Kabuki syndrome 3. Popliteal pterygium syndrome
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15
Q

Premature exfoliation of the mandibular incisors may be seen in

A

hypophosphatasia

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16
Q

Beaten metal/copper skull is seen in which 3 conditions?

A

-Crouzan Syndrome (beaten-metal) -Apert syndrome (beaten metal) -Hypophosphatasia (beaten-copper)

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17
Q

“Teeth floating in air” + skin plantar/palmar hyperkeratosis

A

Papilon-Lefevre syndrome

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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

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19
Q

Ghost teeth on radiology enameloid conglomerates on histo

A

regional odontodysplasia

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20
Q

Black coated tongue cause

A

Bismuth staining

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21
Q

Skull radiology: best diagnosis for punched out radiolucent lesions in a child vs an older adult

A

child: LCH adult: multiple myeloma

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22
Q
A

Hydroxychloroquine pigmentation (antimalarial medication also given to lupus patients)

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23
Q

Lateral Calcifications seen on PAN DDX (5)

A
  1. tonsilith
  2. sialolith
  3. phlebolith
  4. dystrophic acne
  5. calcified lymph node
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24
Q

Globodontia (aka globe shaped teeth), hearing loss, and ocular colobomas are classic of what syndrome?

A

otodental syndrome

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25
Q

Papillary lesion of the soft palate DDX

A
  1. squamous papilloma 2. verruciform xanthoma 3. Giant cell fibroma 4. Sialadenoma papilliferum
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26
Q

Munro’s abscesses

A

psoriasis

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27
Q

Pautriers abscesses

A

Mycosis Fungoides

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28
Q

HLA-Cw6

A

psoriasis

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29
Q

Tissue culture pattern on histology

A

nodular fasciitis

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30
Q

DDX for mixed radiolucent-radiopaque lesion (6) (of course site and appearance play a factor)

A

CEOT

Ossifying fibroma

COC

odontoma

ameloblastic fibro-odontoma

AOT

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31
Q

Late stage florid COD- how do you rule out Pagets disease?

A

There should be no expansion in COD and ALK phos levels are normal (elevated in Pagets)

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32
Q

variant of ameloblastoma with mixed radiolucent/radiopaque appearance that resembles BFOL

A

desmoplastic ameloblastoma

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33
Q

A patient is diagnosed with basal cell carcinoma of the gingiva, what is the more likely diagnosis

A

a misdiagnosed ameloblastoma

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34
Q

-unerupted tooth, usually canine -radiolucency frequently extends below the CEJ of the tooth -snowflake calcifications

A

AOT

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35
Q

PTCH1 gene mutation occurs in what 2 entities?

A

OKC/Gorlin syndrome and CEOT

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36
Q

driven snow calcification appearance

A

CEOT

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37
Q

Amyloid-like acellular amorphous material occurring in CEOT is what?

A

odontogenic ameloblast-associated protein (ODAM)

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38
Q

Clear cell histology DDX (5)

A
  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
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39
Q

A defect or groove in the palate may be caused by what odontogenic neoplasm

A

central odontogenic fibroma

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40
Q

Ground substance in an odontogenic myxoma is composed of

A

GAGs: predominantly hyaluronic acid and chondroitin sulfate

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41
Q

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?

A

Osteogenesis imperfecta (brittle bone disease)

-Osteogenesis imperfecta, ehlers-danlos, marfan syndrome

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42
Q

4 Radiographic hallmarks of osteogenesis imperfecta

A
  1. osteopenia
  2. bowing of long bones
  3. multiple fractures
  4. increased number of wormian bones
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43
Q

Increased number of intrasutural bones (wormian bones) in what two conditions

A

-cleidocranial dysplasia -osteogenesis imperfecta

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44
Q

What disease: Spontaneous and progressive destruction of 1 or more bones (give the 5 names please)

What are the histo findings?

A

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

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45
Q

Many basophilic reversal lines within bone creating a characteristic jigsaw puzzle/mosaic appearance is characteristic histology for what?

A

paget’s disease (osteitis deformans)

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46
Q

10 disorders with Multiple giant cell lesions (6 high yield)…What 2 entities have been associated with central giant cell granulomas in the jaw?

A
  • 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
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47
Q

What cell lines are affected by early post zygotic activating mutation in GNAS

A

-Melanocytes, endocrine cells, osteoblasts *if late in embryogenesis only osteoblasts are affected

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48
Q

Monostotic fibrous dysplasia that affects the maxilla and adjacent bones is termed

A

cranio-facial fibrous dysplasia

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49
Q

3 Syndromes associated with polyostotic fibrous dysplasia

A
  1. Jaffe-lichtenstein
  2. Mazabraud
  3. McCune Albright
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50
Q

early presenting sign of polyostotic fibrous dysplasia is pain and long bone deformity- the deformity is specifically called what? (3 names)

A

deformity of the proximal femur = shepards crook deformity, coxa vara, hockey stick deformity (causes leg bowing)

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51
Q

Clinical: anterior mandibular COD with progressive growth

A

expansive osseous dysplasia

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52
Q

WHat diseasE?

  • Diffuse fibro-osseous lesions of the jaws with prominent psammoma bodies
  • bone fragility
  • bowing/cortical sclerosis of the long bones
A

Gnatho-diaphyseal dysplasia GDD1 mutation

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53
Q

What disease?

  • Parathyroid adenoma or carcinoma
  • Ossifying fibromas of the jaws
  • Renal cysts
  • Wilms tumors
A

Hyperparathyroidism jaw tumor syndrome HRPT2 mutation

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54
Q

-Multifocal osteomas -Supernumerary teeth, impacted teeth, odontomas -adenomatous polyps

A

Gardner’s syndrome APC gene chromo 5

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55
Q

Name 4 less frequently mentioned manifestations of Gardners syndrome

A
  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
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56
Q

-Radiology: radiolucent nidus with possible central opacity “target-like” -Clinical: nocturnal pain relieved by NSAIDs

A

osteoid osteoma (should also be less than 1.5-2cm in size)

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57
Q

Clinical: Genital lesions, conjunctivitis, arthritis, geographic tongue What condition? What HLA? Etiology?

A

-Reiter’s syndrome (reactive arthritis) -HLA-B27 -occurs following venereal disease or dysentery

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58
Q

Multiple chondromas are associated with what 2 diseases/syndromes

A
  1. Ollier disease: sporadic chondromatosis with unilateral tendency
  2. Maffucci syndrome: sporadic chondromatosis and soft tissue angiomas
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59
Q

What syndromes increase the risk for osteosarcoma

A

Paget’s disease

Li-fraumeni syndrome

Hereditary retinoblastoma

Rothmund-thomson syndrome

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60
Q

19 year old male

Bx: Non specific vascular proliferation with fibrous CT and chronic inflammatory cells

A

Massive osteolysis

Vanishing bone disease

phantom bone disease

gorham/gorham-stout disease

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61
Q

25 year old male

HX of veneral disease and arthritis

What is the term for associated genital lesions?

associated HLA?

A

Balanitis circinata

HLA-B27

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62
Q

5 year old female

Other findings: Bone surrounding the teeth is lower denisty, hyperplasia of the soft tissue in the area

DX?

Histo findings?

A

Regional odontodysplasia (ghost teeth)

Histo: enameloid conglomerates (also seen in amelogenesis imperfecta)

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63
Q

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?

-

A
  • Apert syndrome
  • Syndactyly
  • Cloverleaf skull
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64
Q

55 year old male recently with new diagnosis and management of HTN

  • DDX?
  • What medications may cause this presentation?
A
  • Medication related gingival hyperplasia vs Leukemic infiltrate
  • (anti-epileptic)Phenyltoin, (immunosuppressant) cyclosporine, calcium channel blockers (amlodipine)
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65
Q

12 year old male

What is the most likely diagnosis?

What are these lesions?

List all of the manifestations of this disease

Mutation?

A

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)
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66
Q
  • 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?
A
  • Port wine stain (nevus flammeus)
  • 8-10%
  • Sturge Weber syndrome
  • GNAQ chromo 9; not inherited
  • Leptomeningeal angiomas, intellectual disability, contralateral hemiplegia, seizures
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67
Q
  • Most likely diagnosis?
  • What is the cause of the lesions?
  • Associated genetics?
  • Histologic buzzword?
A
  • multifocal epithelial hyperplasia (Heck’s disease)
  • HPV 13 and 32
  • HLA DR4
  • Mitosoid cell
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68
Q

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?
A
  • Molluscum contagiosum
  • poxvirus infection MCV
  • HIV/AIDS, darier disease, atopic dermatitis
  • Henderson paterson bodies
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69
Q
  • 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?
A
  • Dermatosis papulosa nigra
  • FGFR3, PIK3CA (actinic lentigo)
  • Leser-Trelat sign: rare phenomenon associated with internal malignancy
  • Irritated sebK aka Inverted follicular keratosis of Helwig
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70
Q

Biopsy of one of these lesions shows sebaceous hyperplasia

  • Name the associated syndrome
  • Name 3 other findings in this syndrome
A

Muirre-Torre Syndrome

-visceral malignancies, sebaceous adenomas and carcinomas (shown in the image), keratoacanthomas

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71
Q

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
A
  • 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
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72
Q
  • 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?
A

-10-35% for typical leukoplakia, 83% for verruciform variants

Mild: ~1-7%

Moderate: ~4-11%

Severe: 20-43%

73
Q

This patient has been using smokeless tobacco for 10 years.

  • What histologic features would be seen on biopsy?
  • Clinical recommendations?
A
  1. hyperkeratotic and acanthotic
    - intraceullar vacuoles
    - parakeratin chevrons
    - amorphous subepithelial material in the superficial CT

*dysplasia is uncommon

  1. Habit cessation- lesion should go away in ~ 2 weeks, if persists 6 weeks+ then it should be biopsied
74
Q
  • Name 3 inherited syndromes associated with multiple of the lesion depicted in this image
  • Name one acquired cause
A

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
Q

Multiple basal cell carcinomas are common in what inherited genetic disorders?

A
  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
Q

DX?

A

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
Q

Most commonly associated syndrome with this clinical presentation?

What other syndromes also have this lesion?

A

Port wine stain (nevus flammeus)

  • Sturge Weber syndrome
  • Beckwith-Weidemann syndrome
78
Q

What is the term for this clinical presentation?

What are 5 causes?

A
  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
Q

Most likely diagnosis?

  • Key radiographic features
  • Key non-radiographic features
  • What may have a similar presentation and confuse you
A

Segmental odontomaxillary dysplasia

  • missing premolar, vertical oriented coarse bony trabeculae, smaller maxillary sinus
  • Becker nevus, gingival hyperplasia in the area
  • Fibrous dysplasia
80
Q

All lesions with BRAF mutations

A

Some ameloblastomas

50% of Langerhan cell histiocytosis

Melanoma

Acquired melanocytic nevus

81
Q

The HLAs:

  • Cw6
  • B27
  • DR4
  • DR3/B8
  • DRw52
  • B51
  • DRB1
A

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
Q

Mikulicz cells

Mikulicz disease

Mikulicz syndrome

Mikulicz aphthae

A

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
Q

Things to see with a wood’s lamp

A
  • 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
Q

Some serologies:

Diffuse cutaneous systemic sclerosis

Limited cutaneous systemic sclerosis (includes CREST)

Paget’s disease

Lupus Erythematous

A

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
Q

DX?

A

Lichen planus

86
Q

2 clinical syndromes of Acute Sarcoidosis

A

Lofgren syndrome:

Erythema nodosum

Bilateral hilar lymphadenopathy

Arthralgia

Heerfordt syndrome (uveoparotid fever)

Parotid enlargement

Anterior uveitis

Facial paralysis

Fever

87
Q

What is the term for this histologic finding?

Diagnosis?

A

Asteroid body of sarcoidosis

-entrapped fragments of collagen within the granuloma

88
Q

DX?

Name of the entity depicted?

A

Sarcoid granuloma

-Schumann body

89
Q

What is the official / “more appropriate” name for the pattern seen on sialography of Sjogren’s Syndrome?

A

punctate sialectasia (fruit-laden branchless tree)

90
Q

Most likely diagnosis?

What is the etiology?

What is the clinical term applied to these lesions?

A

Bullous impetigo

Typically staph aureus only

Lacquer

91
Q

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?

A

Non bullous impetigo

DDX: HSV, exoliative chelitis(though not really in this picture)

Corn flakes glued to the surface of the lesion

92
Q

What is the most likely diagnosis?

What is the etiology?

What two characteristic clinical terms are applied to this lesion?

A

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
Q

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?

A

Impetigo

Ecthyma

The lesion heals with a permanent scar- scarring is not expected in other lesions of impetigo either bullous or nonbullous

94
Q

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?

A
  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
Q

Name that outdated and terrifying testing method:

Sterilized sarcoid tissue is injected into the skin of a patient

A

Kveim test

96
Q

Olfactory neuroblastoma:

  1. Name for low grade pseudo-rosette
  2. Name for high grade true rosette
A
  1. pseudo-rosette: Homer-Wright
  2. true-rosette: Flexner-Wintersteiner
97
Q

Diagnosis?

A

Congenital syphilis

98
Q

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?

A

Argyll Robertson pupils

Tertiary syphillis

99
Q

Loss of coordination of movement in tertiary syphillis is termed?

A

tabes dorsalis

100
Q

What is the diagnosis?

What other 2 clinical findings are part of the Hutchinson Triad?

A

Hutchinson teeth of Congenital syphilis

-Eigth nerve deafness, interstitial keratitis

101
Q

BUGS summary:

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
A

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
Q

This patient has anesthesia and loss of sweating of the overlying skin in these lesions. Biopsy demonstrated well formed granulomatous inflammation

Diagnosis?

A

Tuberculoid aka paucibacillary leprosy

103
Q

Diagnosis?

What is the term for the characteristic facies?

What would you expect to see on biopsy?

A

Lepromatous (multibacillary) leprosy

  • Leonine facies
  • Sheets of lymphocytes with vacuolated histiocytes (aka lepra cells)
  • No well formed granulomas
104
Q

What is the general term for this clinical presentation?

What is the differential diagnosis?

A

Macrocheilia

105
Q

Children with red teeth DDX

A

Congenital erythropoietic porphyria aka Gunther disease

Leprosy

106
Q

What is the name for the cells depicted in this image?

What is the diagnosis?

A

Lepra cells of lepromatous leprosy- they are vacuolated macrophages

107
Q

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?

A

Pastia lines

Scarlet fever

-White strawberry tongue then red strawberry tongue

108
Q

Bacterial species of chronic periodontitis red complex

A
  • Treponema denticola
  • Tannerella forsythensis
  • Porphyromonas gingivalis
109
Q

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?

A
  • Actinomycosis
  • mimicked by Botryomycosis (usually caused by staph aureus)
  • Definitive diagnosis is Actino
110
Q

This patient also has hypoparathyroidism and hypoadrenocorticism.

Diagnosis?

Associated genetic mutation?

A

APECED: autoimmune polyendocrinpathy-candidasis-ectodermal dystrophy syndrome

  • mucocutaneous candidiasis, nail dystrophy, corneal keratopathy, hypoparathyroid/hypoparathyroid
  • childhood onset
  • Mutation in AIRE gene
111
Q

These are two different presentations of the same disease.

What is the terminology used to describe each image?

What is the diagnosis?

A

Luetic glossitis = atrophic form

Interstitial glossitis = gummatous form

Tertiary syphilis

112
Q

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?
A

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
Q

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?
A

-Gingival Fibromatosis

  • 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
Q

High yield genetic disorders associated with periodontitis in kids/young adults

What is the specific terminology applied to this?

A

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
Q

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

-A. Actinomycetemcomitans (Aggregatibacter actinomycetemcomitans)

-Papilon Lefevre

Bacteria types expand when patients have generalized type and include the red complex

116
Q

What is the term for the histologic feature seen in this image?

What conditions/diseases can this be found in?

A

Hamazaki-Wesenberg Bodies aka yellow-brown bodies

myriad medical conditions: appendicitis, cirrhosis, lymphoid tumours, colon carcinoma and numerous others, most famously sarcoidosis

117
Q

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
A

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)

118
Q

Direct immunofluorescence pattern for Chronic ulcerative stomatitis?

Indirect immunofluorescence finding?

A
  • speckled pattern of IgG deposits in the basal one-third of the epithelium
  • Indirect: presence of stratified epithelium specific anti-nuclear antigen (SES-ANA)
119
Q

What is the characteristic cell depicted here?

What is this diagnostic of?

A

Floret cell

Pleomorphic lipoma

120
Q

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

A

SAPHO syndrome

Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis

121
Q

Multiple bone involvement by primary chronic osteomyelitis in children is termed

A

CRMO: Chronic recurrent multifocal osteomyelitis

thought to be a widespread variant of primary chronic osteomyelitis

122
Q

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?

A
  • 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

-

123
Q

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?

A

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
Q

DX?

A

Calcific metamorphosis and aseptic pulpal necrosis

125
Q

What are the two major clinical findings?

What two conditions produce these radiographic findings?

A
  • Thistle tube/flame shaped pulp chambers & pulp stones
  • Dentin dysplasia type 2 and pulpal dysplasia
126
Q

What termed is applied to this radiographic finding?

What is the diagnosis?

What is the histologic description of the root of these teeth?

A

Rootless teeth

Dentin dysplasia type 1

Histo buzz:

  • whorls of tubular dentin
  • “stream flowing around boulders”
127
Q

What two pathoses can cause a “bull neck” appearance

A

Diptheria

Ludwigs angina

128
Q

Whats my keratin?

Epidermolysis Bullosa simplex

White sponge nevus

Pachyonychia congenita

Oral lesions of pachyonychia congenita

Pachyonychia congenita with neonatal teeth

A

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

129
Q

At least 4 conditions associated with premature tooth loss

A

Acrodynia (pink disease) mercury exposure children

Hypophosphatasia

Dentin Dysplasia type 1 (rootless teeth)

Papilon Lefevre

Haim Munk

Acatalasia

Cohen Syndrome

Ehlers-Danlos

130
Q

What clinical features are depicted in this image?

What is the most likely diagnosis?

What mutation and inheritance pattern is associated with this condition?

A

-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

131
Q

Time to dominate Lipid Reticuloendothelioses….

What are the three diseases & their subtypes?

A
  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)
132
Q

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?

A

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

133
Q

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?

A

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

134
Q

Tay-Sachs:

How does it compare to the other lipoidendoreticuloses?

What enzyme is missing? What accumulates?

What cell type is affected?

A

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)

135
Q

Infant with bilateral facial swellings, fever, leukocytosis, and hyperostosis of his facial bones. Blood cultures negatve. What’s the most probable diagnosis?

A

Caffey Disease (AD, a COL1A1 mutation has been found)

136
Q

Name the skin lesion…What systemic condition is it associated with?

A

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.

137
Q

What are the 3 main components of RAMON syndrome?

A
  1. Cherubism
  2. Gingival fibromatosis
  3. seizures
138
Q

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?

A
  • Multiple large congenital nevi
  • Neurocutaneous melanosis
  • Potentially fatal condition in which patients have multiple or large congenital nevi + melanocytic neoplasms of the CNS (melanomas)
139
Q

What is the term for areas indicated by the yellow arrows?

What diagnosis is this feature specific for?

What mutation is associated?

A
  • Kamino bodies
  • Spitz nevus
  • HRAS
140
Q
  • 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?

A
  • 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
Q

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?
A
  • 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
142
Q

This biopsy is from the posterior lateral tongue.

Diagnosis?

  • is this a neoplastic process?
A
  • 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
Q

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?
A
  • 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
Q

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?
A

Intravascular papillary endothelial hyperplasia (Massons tumor)

  • Non neoplastic/reactive process
  • Dilated vascular spaces, fibrin deposition, endothelial cell proliferation
145
Q

6 year old patient, Diagnosis?

  • Characteristic finding on electron microscopy?
  • stains?

What classification system is currently used?

A
  • 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
Q

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?

A
  • Marfan syndrome
  • Fibrillin-1
  • TGF-Beta
  • Autosomal dominant
  • MEN2B, NBCCS
147
Q

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?
A
  • 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
Q
A
149
Q

Diagnosis?

Term for the foot manifestation?

Oral findings?

A
  • Arsenic poisoning
  • Black foot disease
  • necrotizing ulcerative stomatitis, excessive salivation,
150
Q

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?

A

Chrysiasis: when patients are treated with gold therapy they can develop this slate blue pigmentation in areas exposed to sunlight

-Oral mucositisi

151
Q

What bacteria is most commonly responsible for Lemierre syndrome?

A

Fusobacterium necrophorum

  • usally occurs in teens
  • can be life threatening
  • basically an oropharyngeal infection that can lead to sepsis
152
Q

A newborn is born with jaundice. If the baby is not treated these may develop ______ a dangerous accumulation of bilirubin in the brain

A

Kernicterus

153
Q

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?
A
  • 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
Q

Diagnosis?

A

-Cholesteatoma

155
Q

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?

A
  • Frey’s syndrome: results from injury of the salivary gland
  • Starch iodine test
156
Q

Local causes of delayed tooth eruption

Systemic causes of delayed tooth eruption

A

Local causes: gingival fibromatosis, clefts, ROD, SOD, supernumeraries, tumors

Systemic: anemia, celiac, cerebral palsy, chemotherapy, endocrine disturbances, vit D resistant rickets

157
Q

DX?

What intraoral finding is found in 92% of these patients

A

Rubinstein-Taybi

Talon cusp

158
Q
A

Dens en dente aka dens invaginatus

159
Q

DX?

What might this be caused by?

A

buccal bifurcation cyst

-cervical enamel extension

160
Q

What syndrome would you suspect?

What are the other associated findings?

A

PHACES

P: posterior fossa brain anomalies

H: Hemangioma

A: arterial anomalies

C: Cardic defects

E: Eye anomalies

S: sternal cleft, supraumbilical raphe

161
Q

What is the term for this finding?

Name an associated syndrome?

A

sternal cleft

PHACES

162
Q

What is the term for this radiographic finding?

What is it characteristic of?

A

tram-track or tram-line calcifications

Sturge weber syndrome

163
Q

Salt fish diet –>

Leather and wood-working –>

A

salt fish = sinonasal adenocarcinoma

leather and wood-working = intestinal type sinonasal adenocarcinoma

164
Q

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?

A

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
Q

DX?

A

Cheilitis glandularis

166
Q

What conditions can produce complaints of excess drooling?

If there is no obvious cause, and they get episodic sialorrhea, what is the term?

A
  • cerebral palsy, down syndrome, ALS, parkinsons, heavy metal poisoning, rabies
  • idiopathic paroxysmal sialorrhea
167
Q

Rose bengal dye test is used for?

What other test is used for the same purpose?

A
  • diagnosis of keratoconjunctivitis sicca
  • Schirmer test
168
Q

Difference between Epstein Pearl vs Bohn Nodule vs Ging Cyst of Newborn

A

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
Q

Conditions that affect the nails (8)

A

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
Q

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?

A

osteoporosis circumscripta- occurs in early stage disease

171
Q

What 3 disease cause Forcheimer spots

A

German measles (rubella)

Scarlet fever

Mono

172
Q

Lesion is firm

BX demonstrates benign appearing bone and cartilage

A

Cutright lesion AKA Reactive osseous and chondromatous metaplasia

173
Q

Diagnosis?

Recommended therapy?

A

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
Q

DX?

Tx?

A

DX: Exfoliative chelitis

TX:

  1. Elimination of habit
  2. Tx of any superimposed infection: antiobiotic and or antifungal
  3. topical tacrolimus
175
Q

DX?

TX?

A

DX: transient ligual papillitis

TX: self resolving though you can give topical corticosteroids or analgesics for discomfort

176
Q

Differential DX?

Ancillary testing to confirm dx?

A

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
Q

Describe the finding being depicted in this image. What systemic condition(s) is this typical of?

A

Loss of lamina dura- generalized

  • Hyperparathyroidism, osteomalacia, pagets disease, fibrous dysplasia
178
Q

In addition to the finding depicted this male child has the following bloodwork:

  • Hypophosphatemia
  • Low serum calcium

What is the most likely diagnosis?

What characteristic finding is seen on histology of one of these teeth?

A

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
Q

What is this image depicting and where is this commonly found?

A

Hyaline body aka pulse granuloma

Walls of inflammatory cysts

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