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Andrews/Bolognia Review questions > 03-Blistering Diseases > Flashcards

03-Blistering Diseases Flashcards

1
Q
  1. Name two cadherins in the skin.
A
  1. desmoglein and desmocollin
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2
Q
  1. Name 5 members of the Plakin family.
A
  1. Desmoplakin, BPAg1, Plectin, Envoplakin, Periplakin
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3
Q
  1. Name 2 other names for laminin 5.
A
  1. Epilegrin and Laminin 332
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4
Q
  1. Where are the anchoring filaments located?
A
  1. Lamina Lucida
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5
Q
  1. Where are the anchoring fibrils located?
A
  1. Sublamina densa
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6
Q
  1. What is the predominant collagen in the Lamina densa?
A
  1. Type IV collagen
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7
Q
  1. What is collagen which attaches Type I and III collagen to the Lamina densa?
A
  1. Type VII collagen
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8
Q
  1. Name the tissue used for indirect IF for Pemphigus Vulgaris (and DH).
A
  1. Monkey Esophagus
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9
Q
  1. Name the tissue used for indirect IF for Paraneoplastic Pemphigus.
A
  1. Rat Bladder
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10
Q
  1. Name the tissue used for indirect IF for Pemphigus Foleacius.
A
  1. Guinea Pig Esophagus
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11
Q
  1. Name the medium used for transport of tissue used for DIF.
A
  1. Michel’s media
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12
Q
  1. What is the sign that on slight rubbing the skin reveals the dermis?
A
  1. Nikolsky sign
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13
Q
  1. What is the sign: pressure on the blister surface results in lateral spread?
A
  1. Asboe-Hansen sign
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14
Q
  1. Where do blisters/erosions commonly first appear in pemphigus vulgaris?
A
  1. Mouth
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15
Q
  1. HLA associated with PV?
A
  1. HLA DR4 and DR6 most commonly
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16
Q
  1. The mucosal variant of PV is due to what antibody?
A
  1. Anti-Desmoglein 3
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17
Q
  1. The Mucocutaneous variant of PV is due to what antibody?
A
  1. Anti-Desmoglein 1 & 3
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18
Q
  1. What do you see on pathology in PV?
A
  1. Suprabasilar acantholysis with Tombstoning and eosinophilic spongiosis
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19
Q
  1. What is the DIF pattern in PV?
A
  1. Chickenwire with IgG4
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20
Q
  1. What are the 2 subtypes of Pemphigus Vegetans?
A
  1. Neuman (MC and severe), and Hallopeau (mild, pustular)
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21
Q
  1. Does Pemphigus Foliaceus have oral lesions?
A
  1. NO
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22
Q
  1. Name drugs implicated in Pemphigus Foliaceus
A
  1. Thiol-drugs (captopril, penicillamine, gold thiosulfate), sulfa-releasing, penicillamine, nifedipine
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23
Q
  1. What’s the histology of PF look like?
A
  1. Subcorneal/ Intragranular acantholysis with “cling ons”
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24
Q
  1. In which country is endemic pemphigus found?
A
  1. Brazil
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25
Q
  1. What age population is involved in endemic pemphigus?
A
  1. Children and young adults
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26
Q
  1. What arthropod bite is possibly related to Fogo Selvalgem?
A
  1. Simulium nigrimanum
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27
Q
  1. What is another name for Pemphigus Erythematosus and what other disease does it share features with?
A
  1. Senear-Usher syndrome and Lupus
28
Q
  1. What is the antibody in Pemphigus Erythematosus?
A
  1. Anti-desmoglein 1
29
Q
  1. What are the antibodies in Paraneoplastic Pemphigus?
A
  1. Plakin, BPAg1, Desmoplakin 1 and II, Envoplakin, Periplakin, alpha-2-macroglobulin-like-1
30
Q
  1. Name the associated malignancies with paraneoplastic pemphigus.
A
  1. NHL (40%), CLL (30%), Castleman’s disease (10%), Thymoma/Sarcoma/Waldenstrom’s (6% each)
31
Q
  1. What is the MC malignancy in adolescents with paraneoplastic pemphigus?
A
  1. Castleman’s disease
32
Q
  1. What is the HLA associated with Paraneoplastic pemphigus?
A
  1. HLA-DRB1
33
Q
  1. What is the most consistent symptom of paraneoplastic pemphigus?
A
  1. Intractable Stomatitis
34
Q
  1. What process is associated with poor prognosis in paraneoplastic pemphigus?
A
  1. Bronchiolitis obliterans
35
Q
  1. What are the 2 types of IgA pemphigus and their antibodies?
A
  1. Subcorneal pustular dermatosis (desmocollin 1) and Intraepidermal neutrophilic dermatosis (?Desmoglein 1/3)
36
Q
  1. What are the antibodies in Bullous Pemphigoid?
A
  1. BPAg 1 (230kD) and 2(180kD)
37
Q
  1. What is the HLA assoc with BP?
A
  1. HLA-DQB1
38
Q
  1. What % of BP have oral involvement?
A

37.20%

39
Q
  1. What is seen on DIF in BP?
A
  1. Linear C3 and IgG4 at basement membrane
40
Q
  1. Where does the IIF localize to ?
A
  1. Roof
41
Q
  1. Where does the BPAg2 Ab attack?
A
  1. NC16a domain
42
Q
  1. What common drug is known to cause BP?
A
  1. Furosemide
43
Q
  1. What trimester does pemphigoid gestationis usually occur in?
A
  1. 2nd
44
Q
  1. What is the antibody in pemphigoid gestationis and what domain?
A
  1. BPAg2 and NC16a domain
45
Q
  1. What disease is associated with Pemphigoid gestationis and HLA association?
A
  1. Grave’s and HLA DR3 and DR4
46
Q
  1. What areas of the body are usually spared in PUPPP?
A
  1. Periumbiical, upper chest, face, mucous membranes, palms and soles
47
Q
  1. What is the risk to mother and fetus in PUPPP?
A
  1. None
48
Q
  1. What lab abnormality can Impetigo Herpetiformis be associated with?
A
  1. Hypocalcemia (hypoparathyroidism)
49
Q
  1. Pregnant women with cholestasis of pregnancy are at risk for what process postpartum and why?
A
  1. Postpartum hemorrhage due to low vitamin K (Fetus also at risk)
50
Q
  1. Cicatricial pemphigoid involves the conjunctiva and oral mucosa in what %?
A
  1. 66% and 90% respectively
51
Q
  1. Skin lesions are seen in what % in cicatricial pemphigoid?
A

50.25%

52
Q
  1. What is the name of cicatricial pemphigoid on the head and neck without oral involvement?
A
  1. Brunsting-Perry
53
Q
  1. What is the antibody in cicatricial pemphigoid with mucosal and skin lesions?
A
  1. BPAg2 (distal C-terminal)
54
Q
  1. Ab with ocular cicatricial pemphigoid?
A
  1. Beta-4 integrin
55
Q
  1. Ab with malignancy associated cicatricial pemphigoid?
A
  1. Laminin 332
56
Q
  1. What is the drug of choice for cicatricial pemphigoid with rapidly progressive eye disease?
A
  1. Cyclophosphamide
57
Q
  1. What drug is ineffective for cicatricial pemphigoid with severe eye involvement?
A
  1. Cyclosporine
58
Q
  1. What is the antibody against in Epidermolysis Bullosa Acquisita?
A
  1. Type VII collagen (NC1 domain)
59
Q
  1. What is another name for Dermatitis Herpetiformis?
A
  1. Duhring Disease
60
Q
  1. What areas of the body are involved in DH?
A
  1. Extensor elbows/knees, scalp, and nuchal areas
61
Q
  1. What is the HLA association in DH?
A
  1. HLA DQ2 and DQ8
62
Q
  1. What diseases are associated wit DH?
A
  1. Thyroid disease (38-50%), small bowel T-cell lymphoma; DM, Addison’s, hepatitis, alopecia areata, MG, sarcoidosis, scleroderma, Sjogren’s, SLE, vitiligo
63
Q
  1. What is the appearance of the DIF in DH?
A
  1. Granular IgA along DE junction
64
Q
  1. What are common antibodies are seen in DH?
A
  1. Antiendomysial Ab, Antiepidermal transglutaminase Ab, Antigliadin Ab
65
Q
  1. What drug is commonly known to cause Linear IgA disease?
A
  1. Vancomycin
66
Q
  1. What is the antibody seen in Linear IgA disease?
A
  1. 97-120kD epitope of BPAg2
67
Q
  1. What can flare Grover’s disease?
A
  1. Heat or bedrest (hospitalization)

Andrews/Bolognia Review questions (9 decks)

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