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Flashcards in Blistering Diseases Deck (77)
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0
Q

BP Ag2 (97-120 kDa) antigen

A

Linear IgA disease

1
Q

Bullous pemphigoid antigens

A

Ag1: BP230 (intermediate filaments to HD plaque)

Ag2: BP 180 (transmembrane protein attaches to laminin 5)

Hemidesmosomal proteins

2
Q

Herpes gestationes antigen

A

BP Ag2-NC16A antigen (less often BP Ag1)

3
Q

Circatricial pemphigus antigen

A

BP Ag2, BPAg1, LAM 5, LAM 6

4
Q

Junctional epidermolysis bullosa with pyloric atresia Antigen

A

Alpha6-beta4 integrity

5
Q

Epidermolysis bullosa simplex with muscular dystrophy antigen

A

Plectin

6
Q

Epidermolysis bullosa aquisita and Bullous LE Antigen

A

Collagen VII

7
Q

Diseases with immunofluorescence on roof of salt split skin (4)

A

Bullous pemphigoid
Pemphigoid gestationis
Linear IgA bullous dermatosis
Cicatricial pemphigoid

8
Q

Diseases with immunofluorescence on base of salt split skin

A

Anti-epilgrin cicatricial pemphigoid
Epidermolysis bullosa aquisita
Bullous LE

9
Q

Defect in Collagen VII

A

EBA, DEB, Bullous SLE, CP

10
Q

Name two cadherins in the skin

A

desmoglein and desmocollin

11
Q

Name 5 members of the Plakin family

A

Desmoplakin, BPAg1, Plectin, Envoplakin, Periplakin

12
Q

Name 2 other names for laminin 5

A

Epilegrin and Laminin 332

13
Q

Where are the anchoring filaments located

A

Lamina Lucida

14
Q

Where are the anchoring fibrils located

A

Sublamina densa

15
Q

What is the predominant collagen in the Lamina densa

A

Type IV collagen

16
Q

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

A

Type VII collagen

17
Q

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

A

Monkey Esophagus

18
Q

Name the tissue used for indirect IF for Paraneoplastic Pemphigus

A

Rat Bladder

19
Q

Name the tissue used for indirect IF for Pemphigus Foleacius

A

Guinea Pig Esophagus

20
Q

Name the medium used for transport of tissue used for DIF

A

Michel’s media

21
Q

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

A

Nikolsky sign

22
Q

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

A

Asboe-Hansen sign

23
Q

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

A

Mouth

24
Q

HLA associated with PV?

A

HLA DR4 and DR6 most commonly

25
Q

The mucosal variant of PV is due to what antibody?

A

Anti-Desmoglein 3

26
Q

The mucocutaneous variant of PV is due to what antibody?

A

Anti-Desmoglein 1 & 3

27
Q

What do you see on pathology in PV?

A

Suprabasilar acantholysis with Tombstoning and eosinophilic spongiosis

28
Q

What is the DIF pattern in PV?

A

Chickenwire with IgG4

29
Q

What are the 2 subtypes of Pemphigus Vegetans?

A

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

30
Q

Does Pemphigus Foliaceus have oral lesions?

A

NO

31
Q

Name drugs implicated in Pemphigus Foliaceus

A

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

32
Q

What’s the histology of PF look like?

A

Subcorneal/ Intragranular acantholysis with “cling ons”

33
Q

In which country is endemic pemphigus found?

A

Brazil

34
Q

What age population is involved in endemic pemphigus?

A

Children and young adults

35
Q

What arthropod bite is possibly related to Fogo Selvalgem?

A

Simulium nigrimanum

36
Q

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

A

Senear-Usher syndrome and Lupus

37
Q

What is the antibody in Pemphigus Erythematosus?

A

Anti-desmoglein 1

38
Q

What are the antibodies in Paraneoplastic Pemphigus?

A

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

39
Q

Name the associated malignancies with paraneoplastic pemphigus

A

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

40
Q

What is the MC malignancy in adolescents with paraneoplastic pemphigus?

A

Castleman’s disease

41
Q

What is the HLA associated with Paraneoplastic pemphigus?

A

HLA-DRB1

42
Q

What is the most consistent symptom of paraneoplastic pemphigus?

A

Intractable Stomatitis

43
Q

What process is associated with poor prognosis in paraneoplastic pemphigus?

A

Bronchiolitis obliterans

44
Q

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

A

Subcorneal pustular dermatosis (desmocollin 1)

Intraepidermal neutrophilic dermatosis (Desmoglein 1/3)

45
Q

What are the antibodies in Bullous Pemphigoid?

A

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

46
Q

What is the HLA assoc with BP?

A

HLA-DQB1

47
Q

What % of BP have oral involvement?

A

20%

48
Q

What is seen on DIF in BP?

A

Linear C3 and IgG4 at basement membrane

49
Q

Where does the IIF localize to in BP?

A

Roof

50
Q

Where does the BPAg2 Ab attack in BP?

A

NC16a domain

51
Q

What common drug is known to cause BP?

A

Furosemide

52
Q

What trimester does pemphigoid gestationis usually occur in?

A

2nd

53
Q

What is the antibody in pemphigoid gestationis and what domain?

A

BPAg2 and NC16a domain

54
Q

What disease is associated with Pemphigoid gestationis and HLA association?

A

Grave’s and HLA DR3 and DR4

55
Q

What areas of the body are usually spared in PUPPP?

A

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

56
Q

What is the risk to mother and fetus in PUPPP?

A

None

57
Q

What lab abnormality can Impetigo Herpetiformis be associated with?

A

Hypocalcemia (hypoparathyroidism)

58
Q

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

A

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

59
Q

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

A

66% and 90% respectively

60
Q

Skin lesions are seen in what % in cicatricial pemphigoid?

A

25%

61
Q

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

A

Brunsting-Perry

62
Q

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

A

BPAg2 (distal C-terminal)

63
Q

Ab with ocular cicatricial pemphigoid?

A

Beta-4 integrin

64
Q

Ab with malignancy associated cicatricial pemphigoid?

A

Laminin 332

65
Q

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

A

Cyclophosphamide

66
Q

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

A

Cyclosporine

67
Q

What is the antibody against in Epidermolysis Bullosa Acquisita?

A

Type VII collagen (NC1 domain)

68
Q

What is another name for Dermatitis Herpetiformis?

A

Duhring Disease

69
Q

What areas of the body are involved in DH?

A

Extensor elbows/knees, scalp, and nuchal areas

70
Q

What is the HLA association in DH?

A

HLA DQ2 and DQ8

71
Q

What diseases are associated wit DH?

A

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

72
Q

What is the appearance of the DIF in DH?

A

Granular IgA along DE junction

73
Q

What are common antibodies are seen in DH?

A

Antiendomysial Ab, Antiepidermal transglutaminase Ab, Antigliadin Ab

74
Q

What drug is commonly known to cause Linear IgA disease?

A

Vancomycin

75
Q

What is the antibody seen in Linear IgA disease?

A

97-120kD epitope of BPAg2

76
Q

What can flare Grover’s disease?

A

Heat or bedrest (hospitalization)