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Clinical Dermatology > Blistering Diseases > Flashcards

Blistering Diseases 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
What are the two types of igA pemphigus? What are their antibody targets?
SPD type: target is desmocollin 1. Intraepidermal neutrophilic type: target is Dsg1 and Dsg 3
26
Does drug-induced pemphigus present as PF or PV or PNP?
Presents as PF> PV 4:1
27
What are the culprits for drug-induced pemphigus?
Thiols(which cause acantholysis directly): penicillamine, ACE-inhibitors, ARBs. PAAT the Pemphigus RAT
28
What are the antigen culprits for Linear IgA Bullous Disease? What do they weight?
LAD-1 (120kD), LAD 97 (97kD)
29
What are the antigen targets for Mucous membrane pemphigoid?
C-terminus of BPAG2
30
What are the antigen targets for ocular-predominant MMP?
beta 4 integrin
31
What are the antigen targets for anti-epilegrin MMP?
laminin 322
32
Does DIF, IIF or ELISA correlate with BP disease activity?
IIF levels do not correlate with BP diseaes activity. ELISA does correlate
33
What are other names for epiligrin?
Laminin 322, Laminin 5, Epiligrin
34
What is anti-epiligrin MMP associated with?
solid organ malignancy: adenocarcinoma
35
What are the areas affected by MMP?
Oral (gingiva, buccal, palate) > conjunctiva
36
What is pemphigoid gestationis associated with?
Choriocarcinoma, hydaidiform mole, graves disease, anti-tyroid antibodies
37
What HLA types is pemphigoid gestationis asociated with?
HLA DR3, HLA DR4
38
What do you see on DIF for pemphioid gestationis?
Linear C3> Linear igG
39
What is pemphigoid gestationis doe to the baby?
Early premature delivery and neonates may develop transient blistering
40
Causes of drug-induced pemphigoid?
Fat abdomens covered by Pemphigoid. Furosemide, Ace-inhibitors, cephalosporins, beta-lactams, penicillamine
41
What is anti-p106 pemphigoid associated with?
SJS/TEN 10= TEN
42
What's the first line treatment for MMP? Second line?
First line is dapsone. Second line is cyclophophamide
43
What are the most common areas for Linear IgA in adults and kids?
Adults: trunk/thigh/groin/buttocks. Kids: face
44
What is EBA associated with?
Crohns Disease/IBD (most common) > multiple myeloma, SLE, RA
45
When does EBA present?
Acquired. IN adults
46
What is the antigen target in EBA?
IgG autoantibodies against NC1 domain of type 7 collagen
47
What are the two types of EBA?
Classic mechanobullous EBA and inflammatory BP-like EBA
48
What does classic mechanobullous EBA look like?
Looks like mild dystrophic EB with erosions on acral and trauma-prone sites. Can result in mitten deformities and milia.
49
What does inflammatory EBA look like?
Looks like BP. Heals without scarring or milia
50
What is the HLA subtype for DH?
HLA-DQ2
51
What diseases is DH associated with?
Hashimoto's thyroiditis, IDDM, pernicious anemia
52
What is the antigentic compoenent of gluten?
gliadin
53
Where is TTG2 located? What is anti-TTG2 IgA antibodies responsible for?
Located in GI lamina propria. Responsible for GI involvement
54
Where is TTG3 located? What is anti-TTG3 IgA antibodies responsible for?
Located in epidermis and dermal papillae. Responsible for skin involvement
55
What is the neoantigen recognized by HLA DQ2 in DH?
TTG2-gliadin complex is a neoantigen
56
What % of patients with DH have symptomatic GI disease?
20%
57
What is the ToC for DH? Does it affect GI/lymphoma risk?
Dapsone. Does not decrease GI/lymphoma risk
58
People with DH should avoid what, which may worsen DH?
iodide ingested or topical
59
When does Darier's disease present?
Puberty
60
What is the mutation in Darier's? What does it encode?
ATP2A2. SERCA2 which defective Ca2+ is sequestered into ER
61
What is the clinical picture for Darier's?
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
What diseases are associated with Darier's?
Epilepsy, intellectual impairement, bipolar and depression
63
Treatment for Dariers?
Systemic retinoids
64
What is the mutation in Hailey-Hailey? What does it encode?
ATP2C1. hSPCA1, which causes defective Ca2+ sequestration in the golgi
65
Does Hailey-Hailey or Darier's have mucosal involvement?
Darier's has mucosal invovlement. Hailey Haily has no mucosal involvement
66
Common causes of coma blisters?
Barbiturates and benzos
67
Where does a friction blister split in the skin?
Intraepi, just under granular layer
68
When does bullous arthropod happen in adults?
In patients with hematologic malignancy (CLL, mantle cell lymphoma, NK/T-Cell lymphoma)
69
Where does delayed postburn/postgraft blisters split in the skin?
at the DEJ, due to the fragility of the new DEJ
70
Drugs that cause phototoxic drug eruptions?
Tetracycylines, quinolones, psoralens

Clinical Dermatology (11 decks)

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