HNG 2024

Question 1

Pyoderma gangrenosum category

Answer 1

an ulcerative disorder that falls into the category of neutrophilic dermatoses

Question 2

The most common associated systemic disorders of pyoderma gangrenosum?

Answer 2

rheumatoid arthritis, inflammatory bowel disease, and other autoimmune and inflammatory conditions. Both solid tumors and hematologic malignancies. Pyoderma gangrenosum is associated with ulcerative colitis in 5% to 12% of cases and is associated with Crohn disease in 1% to 2% of cases.

Question 3

pathogenesis of pyoderma gangrenosum

Answer 3

not fully understood. It is thought to involve genetic mutations, neutrophil dysfunction, and immune/inflammatory dysregulation. Some lesions of pyoderma gangrenosum have been found to have a proliferation of clonal T-cells. In addition, inflammasomes have been postulated to be involved in the neutrophil chemotaxis that occurs in these lesions. Inflammasomes are complexes of receptors that are part of innate immune system signaling. Some cases of pyoderma gangrenosum are associated with a mutation in Janus kinase 2, which is involved in the production of several cytokines. Abnormal cytokine signaling by T cells and macrophages is likely a component of the disease process. Lesions of pyoderma gangrenosum have been found to have increased levels of inflammatory mediators. For example, IL-23 has been found to be increased in lesions of pyoderma gangrenosum. IL-23 is important in activating neutrophils and stimulating IL-17 mediated inflammation

Question 4

Inflammasomes

Answer 4

Inflammasomes are complexes of receptors that are part of innate immune system signaling.

Question 5

Histopathology of pyoderma gangrenosum

Answer 5

The disease is a clinical and histologic diagnosis of exclusion as the histologic features are non-specific. The goal of the biopsy is to exclude other causes of ulceration. Early lesions of pyoderma gangrenosum should show an infiltrate of neutrophils in the dermis. Lesions may demonstrate necrotic dermal vessels. Direct immunofluorescence of biopsy specimens of pyoderma gangrenosum is nonspecific.

Question 6

Sweet syndrome

Answer 6

Sweet syndrome, also called acute febrile neutrophilic dermatosis, is a rare disorder that typically presents with acute-onset tender plaques or nodules, fever, arthralgia, ophthalmologic manifestations, headaches, and rarely, oral or genital lesions.

Question 7

Pathergy

Answer 7

an exaggerated response to a minor skin injury or worsening of an existing wound with minimal insult or trauma. This has a wide array of implications for the patient including debridement and simple wound care. This also makes the decision to perform certain surgeries and procedures much more difficult. Lastly, insults to the skin that would be nearly meaningless for most people can be devastating for some patients with pyoderma gangrenosum.

Question 8

Major Criteria of pyoderma gangrenosum

Answer 8

1. Rapid progression of a painful, necrolytic cutaneous ulcer with an irregular, violaceous, and undermined border.
2. Other causes of cutaneous ulceration have been excluded

Question 9

Minor Criteria of pyoderma gangrenosum

Answer 9

1. History suggestive of pathergy or cribriform scarring
2. Systemic diseases associated with pyoderma gangrenosum
3. Histopathologic findings (sterile dermal neutrophilia, +/- mixed inflammation, +/- lymphocytic vasculitis)
4. Treatment response (rapid response to systemic steroid treatment)

Question 10

differential diagnosis of pyoderma gangrenosum

Answer 10

infectious diseases like mycobacterial infection and deep fungal infections. Also included in the differential are noninfectious diseases such as iododerma or bromoderma. Other causes of ulcers to consider are arterial ulcers and Martorell ulcers. Arterial ulcers may have associated weakened pulses on the corresponding anatomical side.

Question 11

What are the risk factors for oral cancer?

Answer 11

Alcohol, smoking, betel nut, immunosuppression, HPV

Question 12

What are the common sites of oropharyngeal carcinoma?

Answer 12

Underside of tongue and floor of the mouth

Question 13

What are the 3 layers of a tooth?

Answer 13

Enamel, Dentin, Root

Question 14

What is the job of the mouth?

Answer 14

Mastication, taste perception, initial starch digestion, swallowing, speech, facial expression.

Question 15

What role does saliva play in the mouth?

Answer 15

Moistens food, buffers oral contents, protects teeth & mucosa, supplies Ca++ and PO4, neutralize
acid.

Question 16

calcium phosphate

Answer 16

calcium salt of phosphoric acid with a chemical formula Ca3(PO4)2. It is also known as Calcium phosphate tribasic or Tricalcium Phosphate. Calcium phosphate stones account for approximately 15% of kidney stone disease. Calcium phosphate stones tend to grow in alkaline urine, especially when Proteus bacteria are present.

Question 17

betel quid

Answer 17

The most common method of using betel nut is to slice it into thin strips and roll it in a betel leaf with slaked lime (powder) or crushed seashells. This leaf package is known as a betel quid, betel nut chew, betel chew, betel pan or betel paan (India)

Question 18

What is CREST syndrome?

Answer 18

calcinosis cutis, Raynaud’s phenomenon, Esophageal dysmotility, sclerodactyly and telangiectasia,

Question 19

En coup de sabre

Answer 19

a rare subset of linear morphea. It usually presents as a pan-sclerotic plaque over the frontoparietal scalp with surrounding scarring alopecia. The patients may have central nervous system involvement in addition to the cutaneous findings

Question 20

Eosinophilic esophagitis histological criteria

Answer 20

Major criteria: ≥ 15 eosinophils per high power field (40x magnification) (Arch Pediatr 2019;26:182)
Minor criteria: extreme basal zone hyperplasia with papillary hyperplasia, eosinophils concentrated in the surface epithelium as opposed to the base, eosinophilic microabscesses, eosinophil degranulation, surface desquamation, lamina propria fibrosis

Question 21

Eosinophilic esophagitis gross description

Answer 21

Major criteria (Gut 2013;62:489)
Mucosa edema
Exudate or white plaques
Longitudinal furrows
Circular esophageal rings (pseudotrachea)
Esophageal stenosis
Minor criteria (Gut 2013;62:489)
Feline esophagus
Concentric mucosal rings observed with some types of motility that disappear with air insufflation
Narrow caliber esophagus
Crêpe paper esophagus
Mucosal laceration or fragility with passage of instruments before dilation

Question 22

Helicobacter heilmannii

Answer 22

Very rare compared to H. pylori
More common in children; due to contact with farm animals or household pets
Helical, 3.5 - 7.5 microns, 0.9 microns in diameter
Long tight spirals; thicker and twice as long as H. pylori and usually visible with H&E stain

Question 23

Autoimmune Hepatitis

Answer 23

ANA, SMA, LKM and/or SLA antibodies
F:M = 7:3
Associated extrahepatic autoimmune disorder: Thyroiditis, Sjogren syndrome, Rheumatoid arthritis.
Hypergammaglobulinemia
Responsive to corticosteroids.
Histo: often sever inflammation and necrosis with abundant plasma cells.

Question 24

Explain stomach acid control

Answer 24

Question 25

Two most common causes of peptic ulcer disease

Answer 25

H pylori NSAIDs

Question 26

two known types of autoimmune hepatitis

Answer 26

Type 1 is distinguished by the presence of anti-smooth muscle antibodies (ASMA) with or without anti-nuclear antibodies (ANA). Type 2 autoimmune hepatitis presents with positive anti-liver/anti-kidney microsome (anti-LMK) type 1 antibodies or anti-liver cytosol (anti-LC) type 1 antibodies.

Question 27

Auerbach plexus

Answer 27

Myenteric plexus, between the inner circular and outer longitudinal smooth muscle layers of the muscularis propria.

Question 28

plexus between the inner circular and outer longitudinal smooth muscle layers of the muscularis propria.

Answer 28

Auerbach plexus. Myenteric plexus.

Question 29

Dieulafoy lesion

Answer 29

an enlarged (1 to 3 mm) submucosal blood vessel that bleeds in the absence of any abnormality such as ulcers or erosions. The majority of the lesions arise in the stomach at the lesser curvature, within 6 cm of the gastroesophageal junction, as this region receives its arterial blood supply directly from the branches of the left gastric artery

Question 30

Histopathology of Dieulafoy lesion

Answer 30

A biopsy is not recommended for the diagnosis of Dieulafoy lesions due to an increased risk of bleeding. Dieulafoy lesion can be distinguished from gastric ulcers on histology by a lack of subintimal fibrosis and mucosal inflammation.

Question 31

Angiodysplasia vs Dieulafoy lesion

Answer 31

Angiodysplasia can be differentiated from Dieulafoy lesions by angiography that reveals the presence of arteriovenous shunting and vascular ectasia; by histological examination of the lesion, which reveals the presence of abnormal submucosal vessels.

Question 32

Dieulafoy lesion

Answer 32

a normal blood vessel with an abnormally wide diameter of 1 to 3 mm, protruding into the mucosa from the submucosa. The protrusion results in a small wall defect in the gastrointestinal tract with fibrinoid necrosis at the base of the lesion; 70% of these lesions are found in the stomach, usually along the lesser curvature.

Question 33

the most common causes of granulomas in liver in the US, and worldwide

Answer 33

sarcoidosis and primary biliary cholangitis being the most common causes in the US, and tuberculosis worldwide.

Question 34

Hepatic granulomas may be classified into four main variants,

Answer 34

caseating, non-caseating, fibrin-ring, and lipogranulomas

Question 35

Hepatic granulomas fibrin-ring variants

Answer 35

Fibrin-ring variants are epithelioid cells in an encircling fibrin ring with a central vacuole. These fibrin-ring granulomas are seen in infections, may be drug-induced, or in Hodgkin lymphoma. Granulomas in Q fever

Question 36

Granulomas in Q fever

Answer 36

Granulomas in Q fever are typically characterized by an eosinophilic fibrinoid ring with a central clear space enclosing a fat vacuole.

Question 37

“pipestem fibrosis”

Answer 37

Schistosomiasis is characterized by progressive portal scarring called “pipestem fibrosis” with marked splenomegaly and variceal hemorrhage.

Question 38

Answer 38

Pipestem fibrosis liver ultrasound, liver schistosomiasis

Question 39

How does the schistosomiasis parasite involve the intestines and bladder?

Answer 39

Adult worms migrate against portal blood flow to the mesenteric venules of the small and large intestine (S. japonicum and S. mekongi), the mesenteric venules of the colon (S. mansoni, S. intercalatum) or the vesical venous plexus (S. haematobium) Migrating eggs elicit a host immune response Egg derived antigens induce a host Th2 immune response, leading to recruitment of eosinophils, granulomatous reaction and later fibrosis

Question 40

What type of parasite is schistosomiasis?

Answer 40

trematode blood fluke

Question 41

trematode

Answer 41

Trematoda is a class of flatworms known as flukes or trematodes.

Question 42

Calcium in sarcoidosis

Answer 42

Sarcoidosis is also associated with elevated angiotensin-converting enzyme levels and hypercalcemia (due to extrarenal production of 1,25-dihydroxy vitamin D), as seen in this patient. PTH is elevated in primary hyperparathyroidism and PTHrP is elevated in malignancy. TSH is elevated in hypothyroidism.

Question 43

best test for TB if granulomas in liver

Answer 43

Polymerase chain reaction (PCR) has a sensitivity of 88% and a specificity of 100% in detecting M tuberculosis.

Question 44

Therapy for primary biliary cholangitis

Answer 44

Polymerase chain reaction (PCR) has a sensitivity of 88% and a specificity of 100% in detecting M tuberculosis.For patients who undergo liver transplantation, outcomes are excellent, with a 1-year survival rate of > 90%. Glucocorticoids are not helpful in the management of primary biliary cholangitis. Penicillamine is used in the treatment of Wilson's disease.

Question 45

rare but fatal complication of intravesical BCG.

Answer 45

Granulomatous hepatitis is a rare but fatal complication of intravesical BCG.

Question 46

Submucosal plexus

Answer 46

Meissner's nerve plexus

Question 47

Crypts of Lieberkuhn

Answer 47

crypts in small intestine

Question 48

Function of Paneth cells in bottom of crypts

Answer 48

Make antimicrobial enzymes (lysozyme and defensins)

Question 49

Function of Brunner Glands

Answer 49

Make alkaline mucus

Question 50

Plicae circulares

Answer 50

Jejunum mucosal folds

Question 51

Celiac Sprue

Answer 51

Autoimmune disease Intolerance to gliadin in wheat Anti-endomysial, anti-tissue transglutaminase, and anti-gliadin antibodies. Histology: blunting and intraepithelial lymphocytes. HLA-DQ2/SQ8. Associated with dermatitis herpetiformis.

Question 52

Whipple Disease. Sex preference

Answer 52

Much more common in males.

Question 53

Cause of Whipple disease

Answer 53

Tropheryma whipplei. Intracellular gram + pathogen Distantly related to Mycobacteria. PAS-positive foamy macrophage.

Question 54

Mickel's diverticulum. Rule of 2's.

Answer 54

Two inches long. 2% of population. 2 feet from ileocecal valve. Presents in kids < 2 years.

Question 55

Pseudomembranous colitis causes

Answer 55

Pseudomembranous colitis results most commonly from C. difficile infections; however, less common etiologies include ischemic colitis, collagenous colitis, inflammatory bowel disease, cytomegalovirus-induced colitis, vasculitis, bacterial and parasitic organisms, Behcet’s Disease, chemotherapeutic medications, and toxins, such as heavy metal poisoning.

Question 56

The most common cause of infectious diarrhea in healthcare settings,

Answer 56

C. difficile causes substantial morbidity and can potentially result in mortality in vulnerable inpatient populations. C. difficile colonization occurs in 13% of hospitalized patients with stays of 2 weeks and up to 50% of patients with stays more than 4 weeks.

Question 57

treatment of C. difficile colitis

Answer 57

Although only oral vancomycin and fidaxomicin have US Food and Drug Administration approval for treatment of C. difficile colitis, metronidazole has remained a first-line agent for four decades. Multiple studies document near the equivalent effect of metronidazole with vancomycin for the initial treatment of mild to moderate C. difficile colitis. Because of superior cure rates for severe disease, administer oral vancomycin as first-line treatment of C. difficile colitis. With recurrent C. difficile colitis after oral vancomycin therapy, consider either fidaxomicin or rifaximin oral administration.

Question 57

esophageal webs / mucosal webs

Answer 57

idiopathic, ledge-like, semi-circumferential mucosal protrusions, can cause non-progressive dysphagia.

Question 58

rings (Schatzki rings)

Answer 58

circumferential protrusions, thicker than webs, involves mucosa, submucosa, plus or minus muscularis propria.

Question 59

Zenker diverticulum

Answer 59

most common diverticula, up of upper esophageal sphincter, elderly, weakness in esophageal wall.

Question 60

epiphrenic diverticulum

Answer 60

immediately above the lower esophageal sphincter, dysfunction of lower esophageal sphincter.

Question 61

histology of achalasia

Answer 61

loss of ganglion cells, hypertrophic nerves in myenteric plexus. achalasia has an increased risk of squamous cell carcinoma

Question 62

etiology of achalasia

Answer 62

most are primary and idiopathic; can be secondary to Chagas disease. achalasia has an increased risk of squamous cell carcinoma.

Question 63

differential of white plaques in the esophagus

Answer 63

Candida esophagitis. Eosinophilic esophagitis. Glycogenic acanthosis. Epidermoid metaplasia

Question 64

histologic criteria for eosinophilic esophagitis

Answer 64

greater than 15 eosinophils per high-power field Eosinophilic microabscesses are greater than 4 eosinophils clustered. More eosinophils near the surface (superficial epithelial clustering)

Question 65

esophagitis dissecans superficialis (sloughing esophagus)

Answer 65

has a healed tissue paper appearance on endoscopy. Histology: split occurs above the basal cell layer, two-tone appearance of squamous epithelium, parakeratosis, minimal inflammation. Older adults, associated with polypharmacy, certain medications.

Question 66

epidermoid metaplasia of the esophagus

Answer 66

esophageal leukoplakia Middle-aged control. Associated with smoking and alcohol Risk of progression to squamous cell carcinoma Histology: compact hyperorthokeratosis with a prominent granular cell layer and flattened rete.

Question 67

kayexalate / sodium polystyrene sulfonate

Answer 67

Question 68

Answer 68

kayexalate / sodium polystyrene sulfonate

Question 69

Answer 69

Bile acid sequestrant

Question 70

Answer 70

Sevelamer / Renvela / Renagel

Question 71

Answer 71

Pill fillers seen in pill esophagitis.

Question 72

histologic features of reflux esophagitis

Answer 72

Basal cell hyperplasia (> 15% of epithelial thickness) Dilated intercellular spaces (spongiosis in derm world) Elongation and congestion of papillae to top third of epithelium. Extravasated red blood cells/vascular lakes. Inflammation (+/- eos, T cells, PMNs). Never out right call "reflux" instead say reflux-type changes.

Question 73

cancer risk of Barrett's esophagus with and without dysplasia

Answer 73

0.2 x 0.5% per year for nondysplastic 0.7% for low-grade dysplasia 7% per year for high-grade dysplasia

Question 74

no established cutoff to make a diagnosis of eosinophilic gastroenteritis. However, the following are the proposed numbers based on reported literature are what?

Answer 74

Stomach: greater than or equal to 30 eosinophils per high-power field in 5 HPF Duodenum: greater than or equal to 30 eosinophils Ileum: more than 56 per HPF in the ileum Right colon: more than 100 per HPF Transverse and descending colon: more than 84 per HPF Rectosigmoid colon: more than 64 per HPF

Question 75

Some of the common diseases associated with eosinophilia include

Answer 75

eosinophilic gastroenteritis, celiac disease, inflammatory bowel disease, infections (parasitic, amebic, fungal), connective tissue disorders, vasculitis, and hypereosinophilic syndrome, for instance. Radiographically, inflammatory bowel disease and lymphoma can mimic eosinophilic gastroenteritis. In the pediatric group, non-IgE mediated food allergy and infantile hypertrophic pyloric stenosis require exclusion, along with differential diagnoses mentioned above

Question 76

Alzheimer's disease

Answer 76

cortex – amyloid plaques and tangles

Question 77

Parkinson's disease (PD)

Answer 77

substantia nigra – Lewy bodies Alpha-synuclein accumulation leading to Lewy Body formation PD with dementia and dementia with Lewy bodies have Lewy bodies in brainstem and cortex

Question 78

Hutington's disease

Answer 78

caudate/putamen –intranuclear inclusions Huntingtin protein with polyglutamine expansion and accumulation of abnormal protein in neurons of caudate and putamen  Trinucleotide repeat Autosomal Dominant with paternal anticipation. Trinucleotide (CAG) repeats expansion in htt gene on chro. 4. Encodes Huntington protien