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Basement membrane/ Skin development maintenance Flashcards

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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

Study These Flashcards
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

Study These Flashcards
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?

Study These Flashcards
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
What are laminins, where are they and which do we need to know?
Laminin 332, 311 and 511 Found in lamina lucida/ densa Disease associations include Junctional EB (LAMC2) Herlitz type EB, generalized atrophic EB, SCC
26
Collagen 7 autoantibody disease associations?
Bullous lupus erythematosus epidermolysis bullosa (NUMEROUS types)
27
for studying
28
29
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
mean age 75-81 yo male \> female **a/w HLA DqB-0301** **Main target = BPAG2 atNC16A domain** *and also BPAG1 via epitope spreading*
30
Which disease process is this?
this is BP but in the early *urticarial phase* *Here we see the **eczematous phase***
31
What is DDx for this\>?
maybe even bullous LP
32
What is going on here?
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
What is this disease?
Pemphigus vegetans
34
What is the mnemonic for drug induced pemphigoid?
PF ChANGs Penicillamine/ PCN derivatives/ photons (radiation) Furosemide Captopril Amoxicillin/ ciprofloxacin NSAIDS Gold Sulfasalazine
35
how is BP and bullous LP similar?
same circulating autoantibodies to BP180 (BPAG2/ collagen 17) *however there is LP like lesions on unaffected areas (blisters)*
36
what is mucous membrane pemphigoid AKA? How many subtypes are there?
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
What do you see in DIF or IIF for pemphigoid diseases?
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
1st line treatment for BP 2nd line etc.
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
Epidermolysis bullosa aquisita epidemiology, disease associations Which **autoantibody?** **Describe the two different forms?**
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
Pemphigus has 3 groups What are they and what are the autoantibody targets?
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
Describe this and what is it
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
What is this?
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
Laminins 111 and 332 interact with \_\_\_\_\_
Integrins a6b4 (Hemidesmosome)
44
Is BPAG1 the primary mediator of autoantibodies for BP?
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
Which gene encodes type 7 collagen and what diseases are associated with nonsense./ missense mutations?
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
The Epidermolysis bullosa acquisita autoantigen (mechanical trauma) / acquired bullous lupus autoantigen
type 7 collagen
47
Spongtiotic dermatitis with Eosinophils in the epidermis differential
HAPPIED herpes gestationis Arthropod bite/ Allergic contact Pemphigoid Pemphigus Incontinentia pigmenti ETox Drug
48
Mounding perakeratosis diff.
PR Seb derm Nummular eczema PRP
49
Superficial epidermal pallor differential
Any nutritional deficiency
50
Irritant contact Derm histo
Superficial pallor Mild spongiosis ulcerations Also think of CNH
51
Incontinentia pigmenti path
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
Collagen 4 is in the
Lamina Densa
53
Which diseases are anti collagen 4 alpha3 Abs present?
goodpastures/ alport

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