03-Blistering Diseases

Question 1

1. Name two cadherins in the skin.

Answer 1

1. desmoglein and desmocollin

Question 2

2. Name 5 members of the Plakin family.

Answer 2

2. Desmoplakin, BPAg1, Plectin, Envoplakin, Periplakin

Question 3

3. Name 2 other names for laminin 5.

Answer 3

3. Epilegrin and Laminin 332

Question 4

4. Where are the anchoring filaments located?

Answer 4

4. Lamina Lucida

Question 5

5. Where are the anchoring fibrils located?

Answer 5

5. Sublamina densa

Question 6

6. What is the predominant collagen in the Lamina densa?

Answer 6

6. Type IV collagen

Question 7

7. What is collagen which attaches Type I and III collagen to the Lamina densa?

Answer 7

7. Type VII collagen

Question 8

8. Name the tissue used for indirect IF for Pemphigus Vulgaris (and DH).

Answer 8

8. Monkey Esophagus

Question 9

9. Name the tissue used for indirect IF for Paraneoplastic Pemphigus.

Answer 9

9. Rat Bladder

Question 10

10. Name the tissue used for indirect IF for Pemphigus Foleacius.

Answer 10

10. Guinea Pig Esophagus

Question 11

11. Name the medium used for transport of tissue used for DIF.

Answer 11

11. Michel’s media

Question 12

12. What is the sign that on slight rubbing the skin reveals the dermis?

Answer 12

12. Nikolsky sign

Question 13

13. What is the sign: pressure on the blister surface results in lateral spread?

Answer 13

13. Asboe-Hansen sign

Question 14

14. Where do blisters/erosions commonly first appear in pemphigus vulgaris?

Answer 14

14. Mouth

Question 15

15. HLA associated with PV?

Answer 15

15. HLA DR4 and DR6 most commonly

Question 16

16. The mucosal variant of PV is due to what antibody?

Answer 16

16. Anti-Desmoglein 3

Question 17

17. The Mucocutaneous variant of PV is due to what antibody?

Answer 17

17. Anti-Desmoglein 1 & 3

Question 18

18. What do you see on pathology in PV?

Answer 18

18. Suprabasilar acantholysis with Tombstoning and eosinophilic spongiosis

Question 19

19. What is the DIF pattern in PV?

Answer 19

19. Chickenwire with IgG4

Question 20

20. What are the 2 subtypes of Pemphigus Vegetans?

Answer 20

20. Neuman (MC and severe), and Hallopeau (mild, pustular)

Question 21

21. Does Pemphigus Foliaceus have oral lesions?

Answer 21

21. NO

Question 22

22. Name drugs implicated in Pemphigus Foliaceus

Answer 22

22. Thiol-drugs (captopril, penicillamine, gold thiosulfate), sulfa-releasing, penicillamine, nifedipine

Question 23

22. What’s the histology of PF look like?

Answer 23

22. Subcorneal/ Intragranular acantholysis with “cling ons”

Question 24

23. In which country is endemic pemphigus found?

Answer 24

23. Brazil

Question 25

24. What age population is involved in endemic pemphigus?

Answer 25

24. Children and young adults

Question 26

25. What arthropod bite is possibly related to Fogo Selvalgem?

Answer 26

25. Simulium nigrimanum

Question 27

26. What is another name for Pemphigus Erythematosus and what other disease does it share features with?

Answer 27

26. Senear-Usher syndrome and Lupus

Question 28

27. What is the antibody in Pemphigus Erythematosus?

Answer 28

27. Anti-desmoglein 1

Question 29

28. What are the antibodies in Paraneoplastic Pemphigus?

Answer 29

28. Plakin, BPAg1, Desmoplakin 1 and II, Envoplakin, Periplakin, alpha-2-macroglobulin-like-1

Question 30

29. Name the associated malignancies with paraneoplastic pemphigus.

Answer 30

29. NHL (40%), CLL (30%), Castleman’s disease (10%), Thymoma/Sarcoma/Waldenstrom’s (6% each)

Question 31

30. What is the MC malignancy in adolescents with paraneoplastic pemphigus?

Answer 31

30. Castleman’s disease

Question 32

31. What is the HLA associated with Paraneoplastic pemphigus?

Answer 32

31. HLA-DRB1

Question 33

32. What is the most consistent symptom of paraneoplastic pemphigus?

Answer 33

32. Intractable Stomatitis

Question 34

33. What process is associated with poor prognosis in paraneoplastic pemphigus?

Answer 34

33. Bronchiolitis obliterans

Question 35

34. What are the 2 types of IgA pemphigus and their antibodies?

Answer 35

34. Subcorneal pustular dermatosis (desmocollin 1) and Intraepidermal neutrophilic dermatosis (?Desmoglein 1/3)

Question 36

35. What are the antibodies in Bullous Pemphigoid?

Answer 36

35. BPAg 1 (230kD) and 2(180kD)

Question 37

36. What is the HLA assoc with BP?

Answer 37

36. HLA-DQB1

Question 38

37. What % of BP have oral involvement?

Answer 38

37.20%

Question 39

38. What is seen on DIF in BP?

Answer 39

38. Linear C3 and IgG4 at basement membrane

Question 40

39. Where does the IIF localize to ?

Answer 40

39. Roof

Question 41

40. Where does the BPAg2 Ab attack?

Answer 41

40. NC16a domain

Question 42

41. What common drug is known to cause BP?

Answer 42

41. Furosemide

Question 43

42. What trimester does pemphigoid gestationis usually occur in?

Answer 43

42. 2nd

Question 44

43. What is the antibody in pemphigoid gestationis and what domain?

Answer 44

43. BPAg2 and NC16a domain

Question 45

44. What disease is associated with Pemphigoid gestationis and HLA association?

Answer 45

44. Grave’s and HLA DR3 and DR4

Question 46

45. What areas of the body are usually spared in PUPPP?

Answer 46

45. Periumbiical, upper chest, face, mucous membranes, palms and soles

Question 47

46. What is the risk to mother and fetus in PUPPP?

Answer 47

46. None

Question 48

47. What lab abnormality can Impetigo Herpetiformis be associated with?

Answer 48

47. Hypocalcemia (hypoparathyroidism)

Question 49

48. Pregnant women with cholestasis of pregnancy are at risk for what process postpartum and why?

Answer 49

48. Postpartum hemorrhage due to low vitamin K (Fetus also at risk)

Question 50

49. Cicatricial pemphigoid involves the conjunctiva and oral mucosa in what %?

Answer 50

49. 66% and 90% respectively

Question 51

50. Skin lesions are seen in what % in cicatricial pemphigoid?

Answer 51

50.25%

Question 52

51. What is the name of cicatricial pemphigoid on the head and neck without oral involvement?

Answer 52

51. Brunsting-Perry

Question 53

52. What is the antibody in cicatricial pemphigoid with mucosal and skin lesions?

Answer 53

52. BPAg2 (distal C-terminal)

Question 54

53. Ab with ocular cicatricial pemphigoid?

Answer 54

53. Beta-4 integrin

Question 55

54. Ab with malignancy associated cicatricial pemphigoid?

Answer 55

54. Laminin 332

Question 56

55. What is the drug of choice for cicatricial pemphigoid with rapidly progressive eye disease?

Answer 56

55. Cyclophosphamide

Question 57

56. What drug is ineffective for cicatricial pemphigoid with severe eye involvement?

Answer 57

56. Cyclosporine

Question 58

57. What is the antibody against in Epidermolysis Bullosa Acquisita?

Answer 58

57. Type VII collagen (NC1 domain)

Question 59

58. What is another name for Dermatitis Herpetiformis?

Answer 59

58. Duhring Disease

Question 60

59. What areas of the body are involved in DH?

Answer 60

59. Extensor elbows/knees, scalp, and nuchal areas

Question 61

60. What is the HLA association in DH?

Answer 61

60. HLA DQ2 and DQ8

Question 62

61. What diseases are associated wit DH?

Answer 62

61. Thyroid disease (38-50%), small bowel T-cell lymphoma; DM, Addison’s, hepatitis, alopecia areata, MG, sarcoidosis, scleroderma, Sjogren’s, SLE, vitiligo

Question 63

62. What is the appearance of the DIF in DH?

Answer 63

62. Granular IgA along DE junction

Question 64

63. What are common antibodies are seen in DH?

Answer 64

63. Antiendomysial Ab, Antiepidermal transglutaminase Ab, Antigliadin Ab

Question 65

64. What drug is commonly known to cause Linear IgA disease?

Answer 65

64. Vancomycin

Question 66

65. What is the antibody seen in Linear IgA disease?

Answer 66

65. 97-120kD epitope of BPAg2

Question 67

66. What can flare Grover’s disease?

Answer 67

66. Heat or bedrest (hospitalization)