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derm > Basement membrane/ Skin development maintenance > Flashcards

Basement membrane/ Skin development maintenance Flashcards

1
Q

from superficial to deep, what are the four subregions of the basement membrane zone

A
  1. cytoskeleton, hemidesmosomal plaques, plasma memranes of basal keratinocytes
  2. lamina lucida (electron lucent region)
  3. lamina densa
  4. sublamina densa containing anchoring fibrils, plaques, filamentous proteins
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2
Q

Elastin, types 1, 3 and 7 collagen are found in which layer

A

sublamina densa

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3
Q

laminin 332 and type 4 collage are found in which region

A

Lamina densa (not sublamina)(

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4
Q

Integrin subunits alpha 6/ beta4 and BPAG2 are found ____

A

lamina lucida

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5
Q

BPAG1, the epidermal isoform of ____,

A

plectin

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6
Q

BPAG1 and plectin interact with the lamina densa via which subunits

A

Integrin subunit a6, b4

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7
Q

BPAG2 is targeted by autoantibodies from patients with ___, _______, ______ and _____

A

BP, Pemphigoid gestationis, MMP and LABD

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8
Q

The autoantibodies of MMP, LABD, PG and BP target BPAG2, the difference with MMP autoantibodies is __

A

it targets the distal carboxy terminus - deeper into the basement membrane

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9
Q

The difference between BPAG1 and BPAG2

A

BPAG2 HD anchoring filament complexes in basal keratinocytes

BPAG1 connects keratinocytes together

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10
Q

BPAG1 and BPAG2 (collagen 17) disease association and genetic disease association (2 total)

A

Bullous pemphigoid

Genetic = recessive EB complex

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11
Q

Type XVII collagen aka _____, is involved in which bullous diseases (4)

A

BPAG2, BP, PG, MMP, linear IgA bullous dermatosis

Bonus: junctional EB (mild)

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12
Q

Which autoimmune disease is associated with Ab’s against integrin subunit B(beta)-4

A

Occular MMP

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13
Q

Auto-abs against type 7 collagen is seen in

A

EB acquisita

Bullous eruption of SLE

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14
Q

Kindler syndrome - autosomal ______

Caused by mutations in ____

Path?

A

Autosomal recessive

FERMT1 gene

trauma induced blisters, photosensitivitry, pokiloderma, variable mucosa involvement, increased risk of mucosal SCC

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15
Q

Pemphigoid gestiationis targets which auto ab

A

BPAG2 180 HD

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16
Q

linear IgA bullous dermatosis ABs target

A

BPAG2 HD

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17
Q

EB Acquisita / bullous eruption of SLE targets ___ (auto antibody)

A

Type 7 collagen

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18
Q

BP auto ab’s

A

BPAG1 and 2

Aka BP230 *BPAG12) and BP180 (BPAG2 aka collagen 17)

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19
Q

skin derived from which embryonic germ layers?

A

Ectoderm -epidermis, Merkel cells, adnexal structures, melanocytes (neural crest derivatives), nerves (neuroectoderm)

Mesoderm - fibroblasts, LCs, vessels, inflammatory cells

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20
Q

Lines of blaschko are thought to represent ____ in organogenesis

A

migration pathways of epidermal cells during embryogenesis

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21
Q

Harlequin icthyosis (HI) is from a deletorious mutation in _____

A

ABCA12

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22
Q

Plectin mutations are associated with which diseases?

hint: three out of the five are the same disease, but different variants

A

Epidermolysis bullosa simplex w/ muscular dystrophy, EB with pyloric atresia, EB Ogna variant

Paraneoplastic pemphigus

Bullous pemphigoid

23
Q

Where are integrins located

Which ones do we need to know (related to a disease)

Talk about their functions

A

Basal keratinocytes, hemidesmosomes

  • in the lamina lucida layer*
  • a6b4 mutations lead to junctional EB with pyloric atresia*

function: structure/ integrity, epidermal homeostasis (adhesion), cell signaling, hair growth

24
Q

Autoantibodies vs collagen 17 = which disease associations?

A

bullous pemphigoid, gestational pemphigoid, mucous membrane pemphigoid, LP pemphigoides, and LABD

here is BP:

  • Vesicles/Bullae on normal or erythematous skin +/- urticarial plaques (annular/figurate pattern)•Symmetrically, flexural areas
  • Blisters 1-3 cm tense, clear fluid or hemorrhagic persist for several days leaving eroded and crusted areas
  • Residual PIH, rarely milia
  • Oral cavity involved in 10-30%•50% patients with peripheral eosinophilia
25
Q

What are laminins, where are they and which do we need to know?

A

Laminin 332, 311 and 511

Found in lamina lucida/ densa

Disease associations include

Junctional EB (LAMC2)

Herlitz type EB, generalized atrophic EB, SCC

26
Q

Collagen 7 autoantibody disease associations?

A

Bullous lupus erythematosus

epidermolysis bullosa (NUMEROUS types)

27
Q

for studying

A
28
Q
A
29
Q

Key features of pemphigoid group of disease

Mean age:

Female vs. male

any allele associations?

Understand the pathogenesis of target antigens → what is main target, then name the other two

A

mean age 75-81 yo

male > female

a/w HLA DqB-0301

Main target = BPAG2 atNC16A domain and also BPAG1 via epitope spreading

30
Q

Which disease process is this?

A

this is BP but in the early urticarial phase

Here we see the eczematous phase

31
Q

What is DDx for this>?

A

maybe even bullous LP

32
Q

What is going on here?

A

Gestational Pemphigoid / pemphigoid gestiationis

rapid onset in 2nd/3rd trimester, associated with graves/ anti-TPO antibodies, choriocarcinoma, hydatiform mole

HLA-DR3, HLA-DR4 or both

  • DIF: linear C3 (100%), linear IgG (30%)*
  • Neonate can develop transient blistering!*
    remember: Pemphigus vulgaris (desmoglein 3) auto ab’s can blister babies too because their skin configuration is essentially mucous membrane adult like
33
Q

What is this disease?

A

Pemphigus vegetans

34
Q

What is the mnemonic for drug induced pemphigoid?

A

PF ChANGs

Penicillamine/ PCN derivatives/ photons (radiation)

Furosemide

Captopril

Amoxicillin/ ciprofloxacin

NSAIDS

Gold

Sulfasalazine

35
Q

how is BP and bullous LP similar?

A

same circulating autoantibodies to BP180 (BPAG2/ collagen 17)

however there is LP like lesions on unaffected areas (blisters)

36
Q

what is mucous membrane pemphigoid AKA?

How many subtypes are there?

A

Cicatricial pemphigoid

Four subtypes:

  1. Ocular CP (antibodies to the b4 subunit of the a6b4 integrin)
  2. Combined mucocutaneous CP (BPAG2 Abs aka BP180)
  3. Mucous membrane only (MMP)
    1. Anti-epiligrin CP (laminin 332 Ab’s)
37
Q

What do you see in DIF or IIF for pemphigoid diseases?

A

DIF → most reliable (80-95% sensitive) linear IgG, IgA and/or C3 along the BMZ

IIF → only 20-30% have detectable circulating auto-antibodies

38
Q

1st line treatment for BP

2nd line etc.

A
  1. systemic steroids and steroid sparing immunosuppressants (cellcept, Imuran, MTX, cyclophosphamide)
  2. tetracyclines, niacinamide
  3. Dapsone for mucosal predominant
  4. Rituximab for treatment resistant cases
  5. IVIG, plasma exchange
39
Q

Epidermolysis bullosa aquisita epidemiology, disease associations

Which autoantibody?

Describe the two different forms?

A

Most common in adults, it is rare and acquired

Associated diseases: Crohns > myeloma, SLE, RA, diabetes, thyroiditis

Target: IgG autoantibodies to collagen 7

Form 1: classic mechanobullous → noninflammatory bullae form on trauma prone sites, heals with atrophic scars, milia and dyspigmentation

Form 2: Inflammatory → clinically indistinguishable from BP/ MMP → heals without scars!

40
Q

Pemphigus has 3 groups

What are they and what are the autoantibody targets?

A

Pemphigus vulgaris - IgG to Desmoglein 3 or both desmoglein 1 and 3

pemphigus foliaceus - IgG to desmoglein 1

paraneoplastic pemphigus - Autoantibodies against Plakin molecules as well as T cell mediated autoimmune reaction leading to interface dermatitis

41
Q

Describe this and what is it

A

Pemphigus foliaceous

Autoantibody to DG1

well demarcated, transient, impetigo like crusted erosions on an erythematous base favoring seborrheic distr. (cornflake scale), no mucosal involvment

42
Q

What is this?

A

Pemphigus erythematosus aka senear usher syndrome

Lupus/ P. foliaceus overlap

Malar region and seborrheic areas

DIF shows intercellular pemphigus pattern + lupus pattern (granular to linear IgG and c3 along BMZ)

sun protection, steroids +/- dapsone

43
Q

Laminins 111 and 332 interact with _____

A

Integrins a6b4 (Hemidesmosome)

44
Q

Is BPAG1 the primary mediator of autoantibodies for BP?

A

No, it is targeted via epitope spreading

BPAG2 is the primary target as it connects Hd with basement membrane

specifically autoantibodies target the NC16A domain

45
Q

Which gene encodes type 7 collagen and what diseases are associated with nonsense./ missense mutations?

A

COL7A1 mutations cause both recessive and dominant forms of dystrophic EB

  • These patients have no anchoring fibrils in their epidermal BM and manifest extreme skin fragility, scars, increased risk for SCCs*
  • Recessive carriers usually only have 1 allele and thus have less fragility*
46
Q

The Epidermolysis bullosa acquisita autoantigen (mechanical trauma) / acquired bullous lupus autoantigen

A

type 7 collagen

47
Q

Spongtiotic dermatitis with Eosinophils in the epidermis differential

A

HAPPIED

herpes gestationis

Arthropod bite/ Allergic contact

Pemphigoid

Pemphigus

Incontinentia pigmenti

ETox

Drug

48
Q

Mounding perakeratosis diff.

A

PR

Seb derm

Nummular eczema

PRP

49
Q

Superficial epidermal pallor differential

A

Any nutritional deficiency

50
Q

Irritant contact Derm histo

A

Superficial pallor

Mild spongiosis

ulcerations

Also think of CNH

51
Q

Incontinentia pigmenti path

A

Dyskeratosis in clumps

Small adnexal structures

Eosinophilic spongiosis

Vesicles early Papillar/

verrucal is stage 2

Pigmented/blaschkoid stage 3

Atrophy/ scarring in stage 4

52
Q

Collagen 4 is in the

A

Lamina Densa

53
Q

Which diseases are anti collagen 4 alpha3 Abs present?

A

goodpastures/ alport

derm (14 decks)

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