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Clinical Dermatology > Blistering Diseases > Flashcards

Blistering Diseases Flashcards

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Dermatology Board Prep flashcards
1
Q

Connections: In adherens junctions,what does the E/P/N-cadherins bind to? What does that bind to?

A

E-, P-, N-cadherins bind to armadillo family (B-catenin, plakoglobin) which bind to alpha-catenin which bind to actin microfilaments

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2
Q

Connections: In desomosomal junctions,what does the dsg and dcg bind to? What does that bind to?

A

Desmosomal cadherins (demoglein and desmocollins) bind to armadillo (plakophilin and plakoglobin) which bind to intracytoplasmic plakins (desmoplakin 1, 2, BPAG, plectin, envoplakin, periplakin) which bind to keratin filaments

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3
Q

How do adherens junctions compare to desmosomes? In Speed and strength?

A

Adherens quick but weak. Desmosomes are slow but strong

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4
Q

Where are desmoglein 1 expressed in the epidermis?

A

At all levels of skin. Not in mucosal epithelium

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5
Q

Where are desmoglein 3 expressed in the epidermis?

A

Throughout mucosal epithelium and in the lower protion of the epidermis

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6
Q

What other diseases is pemphigus vulgaris associated with?

A

Myasthenia gravis, Thymoma, autoimmune thyroiditis

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7
Q

Neonates from Pemphigus vulgaris mothers get PV because?

A

maternal IgG autoantibodoes against Dsg 3 cross placenta, causing transient blistering

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8
Q

How is PV different from Hailey Hailey on histopath?

A

PV has extensive hairfollicle involvement. Also lack epidermal hyperplasia.

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9
Q

Whats the best IIF substrate for PF, PV, PNP?

A

PF: guinea pig, PV: monkey esophagus, PNP: Rat bladder

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10
Q

Whis is DIF?

A

It assess patient’s skin for in vivo bound IgG

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11
Q

What is IIF? What is ELISA

A

Assess patient’s serum for circulating IgG

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12
Q

Does DIF, IIF or ELISA correlate with PV disease activity?

A

IIF and ELISA correlate with diseaes activity?

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13
Q

What is fogo selvagem?

A

Endemic form of PV in Brazil caused by black flies

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14
Q

What is Senear Usher?

A

Pemphigus Erythematosus

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15
Q

What neoplasms is ParaNeoplastic Pemphigus associated with?

A

NHL> CLL>Castleman’s> thymoma> sarcoma > Waldenstrom macroglobulinemia

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16
Q

What are the antibody targets for PNP?

A

Entire plakin family, desmoglein 1, desmoglein 2

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17
Q

What is the most common skin finding for PNP?

A

Lichenoid > pemphigus-like

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18
Q

How is PNP different from PV in terms of skin areas affected?

A

PNP affects the palms and soles

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19
Q

What is the most common cause of death in PNP?

A

underlying malignancy and bronchiolitis obliterans

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20
Q

What are other names of BPAG 1? Is it a member of the plakin family?

A

BPAG 230. A member of the plakin family

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21
Q

What are other names of BPAG 2? Is it a member of the plakin family?

A

BPAG 180. Collagen type 17

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22
Q

Where on BPAG2 is the BP target?

A

Non-collagenous NC 16A domain

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23
Q

How does IgA pemphigus present?

A

IgA pemphigus presents as pusutules in annular pattern in the axillae and groin

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24
Q

IgA pemphigus is associated with what? What is the ToC?

A

Associated with igA gammopathy. Tx with dapsone.

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25
Q

What are the two types of igA pemphigus? What are their antibody targets?

A

SPD type: target is desmocollin 1. Intraepidermal neutrophilic type: target is Dsg1 and Dsg 3

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26
Q

Does drug-induced pemphigus present as PF or PV or PNP?

A

Presents as PF> PV 4:1

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27
Q

What are the culprits for drug-induced pemphigus?

A

Thiols(which cause acantholysis directly): penicillamine, ACE-inhibitors, ARBs. PAAT the Pemphigus RAT

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28
Q

What are the antigen culprits for Linear IgA Bullous Disease? What do they weight?

A

LAD-1 (120kD), LAD 97 (97kD)

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29
Q

What are the antigen targets for Mucous membrane pemphigoid?

A

C-terminus of BPAG2

30
Q

What are the antigen targets for ocular-predominant MMP?

A

beta 4 integrin

31
Q

What are the antigen targets for anti-epilegrin MMP?

A

laminin 322

32
Q

Does DIF, IIF or ELISA correlate with BP disease activity?

A

IIF levels do not correlate with BP diseaes activity. ELISA does correlate

33
Q

What are other names for epiligrin?

A

Laminin 322, Laminin 5, Epiligrin

34
Q

What is anti-epiligrin MMP associated with?

A

solid organ malignancy: adenocarcinoma

35
Q

What are the areas affected by MMP?

A

Oral (gingiva, buccal, palate) > conjunctiva

36
Q

What is pemphigoid gestationis associated with?

A

Choriocarcinoma, hydaidiform mole, graves disease, anti-tyroid antibodies

37
Q

What HLA types is pemphigoid gestationis asociated with?

A

HLA DR3, HLA DR4

38
Q

What do you see on DIF for pemphioid gestationis?

A

Linear C3> Linear igG

39
Q

What is pemphigoid gestationis doe to the baby?

A

Early premature delivery and neonates may develop transient blistering

40
Q

Causes of drug-induced pemphigoid?

A

Fat abdomens covered by Pemphigoid. Furosemide, Ace-inhibitors, cephalosporins, beta-lactams, penicillamine

41
Q

What is anti-p106 pemphigoid associated with?

A

SJS/TEN 10= TEN

42
Q

What’s the first line treatment for MMP? Second line?

A

First line is dapsone. Second line is cyclophophamide

43
Q

What are the most common areas for Linear IgA in adults and kids?

A

Adults: trunk/thigh/groin/buttocks. Kids: face

44
Q

What is EBA associated with?

A

Crohns Disease/IBD (most common) > multiple myeloma, SLE, RA

45
Q

When does EBA present?

A

Acquired. IN adults

46
Q

What is the antigen target in EBA?

A

IgG autoantibodies against NC1 domain of type 7 collagen

47
Q

What are the two types of EBA?

A

Classic mechanobullous EBA and inflammatory BP-like EBA

48
Q

What does classic mechanobullous EBA look like?

A

Looks like mild dystrophic EB with erosions on acral and trauma-prone sites. Can result in mitten deformities and milia.

49
Q

What does inflammatory EBA look like?

A

Looks like BP. Heals without scarring or milia

50
Q

What is the HLA subtype for DH?

A

HLA-DQ2

51
Q

What diseases is DH associated with?

A

Hashimoto’s thyroiditis, IDDM, pernicious anemia

52
Q

What is the antigentic compoenent of gluten?

A

gliadin

53
Q

Where is TTG2 located? What is anti-TTG2 IgA antibodies responsible for?

A

Located in GI lamina propria. Responsible for GI involvement

54
Q

Where is TTG3 located? What is anti-TTG3 IgA antibodies responsible for?

A

Located in epidermis and dermal papillae. Responsible for skin involvement

55
Q

What is the neoantigen recognized by HLA DQ2 in DH?

A

TTG2-gliadin complex is a neoantigen

56
Q

What % of patients with DH have symptomatic GI disease?

A

20%

57
Q

What is the ToC for DH? Does it affect GI/lymphoma risk?

A

Dapsone. Does not decrease GI/lymphoma risk

58
Q

People with DH should avoid what, which may worsen DH?

A

iodide ingested or topical

59
Q

When does Darier’s disease present?

A

Puberty

60
Q

What is the mutation in Darier’s? What does it encode?

A

ATP2A2. SERCA2 which defective Ca2+ is sequestered into ER

61
Q

What is the clinical picture for Darier’s?

A

Warty crusted malodorous papules in a seb distribution. Keratotic palmar papules/pits. Oral cobblestoning. V shaped nicking. Red/white alternating nail streaks. Acrodermatitis verruciformis of Hopf

62
Q

What diseases are associated with Darier’s?

A

Epilepsy, intellectual impairement, bipolar and depression

63
Q

Treatment for Dariers?

A

Systemic retinoids

64
Q

What is the mutation in Hailey-Hailey? What does it encode?

A

ATP2C1. hSPCA1, which causes defective Ca2+ sequestration in the golgi

65
Q

Does Hailey-Hailey or Darier’s have mucosal involvement?

A

Darier’s has mucosal invovlement. Hailey Haily has no mucosal involvement

66
Q

Common causes of coma blisters?

A

Barbiturates and benzos

67
Q

Where does a friction blister split in the skin?

A

Intraepi, just under granular layer

68
Q

When does bullous arthropod happen in adults?

A

In patients with hematologic malignancy (CLL, mantle cell lymphoma, NK/T-Cell lymphoma)

69
Q

Where does delayed postburn/postgraft blisters split in the skin?

A

at the DEJ, due to the fragility of the new DEJ

70
Q

Drugs that cause phototoxic drug eruptions?

A

Tetracycylines, quinolones, psoralens

Clinical Dermatology (11 decks)

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