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Neville and Damm > Chapter 16-Derm Dz 3 > Flashcards

Chapter 16-Derm Dz 3 Flashcards

1
Q

4 broad categories of epidermolysis bullosa

A
  1. simplex
  2. junctional
  3. dystrophic
  4. kindler syndrome
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2
Q

epidermolysis bullosa simplex: disease severity? mutations? (2)

A

mild disease

mutations in keratin 5 and 14

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3
Q

epidermolysis bullosa junctional type: disease severity? mutations? (3)

A

frequently fatal at birth

Mutations coding for: Laminin-332, type XVII collagen, or alpha 6 beta 4 integrin

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4
Q

Dystrophic type epidermolysis bullosa is caused by mutation encoding what protein?

A

type VII collagen

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5
Q

Dominant and recessive forms of dystrophic epidermolysis bullosa are very different diseases- dominant type are ____ severe and recessive types are ___ severe

A

dominant are less severe: disfiguring but typically not life threatening

recessive is more severe- even minor trauma causes bullae

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6
Q

Mittenlike deformity is characteristic of what

A

recessive type dystrophic epidermolysis bullosa

-fingers fuse together from scarring (IF the patient even makes it to the 2nd decade of life)

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7
Q

Oral manifestations of dominant type dystrophic epidermolysis bullosa (2)

A

mild, 1)gingival erythema and tenderness, 2)gingival recession (reduction in depth of buccal vestibule)

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8
Q

Oral manifestations of recessive type dystrophic epidermolysis bullosa (3)

A

1)Bullae and vesicles intraorally from virtually any food with texture → 2)microstomia & 3)ankyloglossia from scarring
Requires soft diet which is cariogenic

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9
Q

Kindler syndrome is a very rare autosomal recessive form of epidermolysis bullosa that results from mutation in _____

A

Kindler syndrome

Mutation in the FERMT1 gene that encodes hemidesmosomal attachment protein, kindlin-1

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10
Q

Where does clefting occur histologically in each type of epidermolysis bullosa

A

Simplex → intraepithelial clefting (think pemphigus?)

Junctional, dystrophic and Kindler → subepithelial clefting (think pemphigoid?)

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11
Q

More specifically where does epithelial clefting occur in Junctional, dystrophic and Kindler type EB

A
  • Junctional: clefting at the level of lamina lucida
  • Dystrophic: clefting at the level of lamina densa
  • Kindler: clefting just below the basal cell layer
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12
Q

____ ____ form of EB is more prone to cutaneous SCC

A

recessive dystrophic

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13
Q

Which form of epidermolysis bullosa most commonly has oral manifestations? Which form is most commonly associated with dental abnormalities?

A

dystrophic for oral manifestations, but junctional for dental abnormalities

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14
Q

As opposed to the patient’s tissue being used in direct immunofluorescence…What tissue is used in indirect immunofluoresence?

A

old world monkey esophagus

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15
Q

What are the 4 types of pemphigus? Which two affect the oral mucosa? Which one is the most common?

A
  1. Pemphigus vulgaris - oral mucosa * most common (vulgaris = common in Latin)
  2. Pemphigus vegetans - oral mucosa (rare, now thought of as a varient of vulgaris)
  3. pemphigus erythematosus
  4. Pemphigus foliaceus
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16
Q

What is the coined phrase for the behavior of the oral lesions in pemphigus vulgaris?

A

“first to show, last to go” (first sign and hard to treat)

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17
Q

What are the two targets for the autoantibodies produced in pemphigus vulgaris? These two targets are components of what critical stuctures?

A

Desmoglein 3 and Desmoglein 1…components of DESMOSOMES

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18
Q

Which desmoglein is preferentially expressed in the parabasal region of the epidermis and ORAL epithelium?

A

Desmoglein 3 (desmoglin 1 has minimal oral expression)

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19
Q

Pemphigus differential: What are 3 medications associated with pemphigus-like oral eruption?

A
  1. penicillamine
  2. ACE inhibitors
  3. NSAIDs
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20
Q

Pemphigus differential: what type of malignancy is most associated with paraneoplastic pemphigus?

A

lymphoreticular malignancies

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21
Q

Pemphigus Vulgaris differential: What are the 3 entities discussed in neville?

A
  1. Pemphigus-like oral eruption 2. Paraneoplastic pemphigus 3.chronic benign familial pemphigus (aka Hailey-Hailey disease)
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22
Q

What is the avg age at dx for Pemphigus vulgaris? Sex predilection? What 3 ethnicities are most prone?

A

50 years, no sex predilection…Mediterranian, South Asian, or Jewish heratage (random AF)

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23
Q

What % of patients have oral lesions of pemphigus vulgaris before cutaneous lesions?…What % of them have oral lesions overall?

A

more than 50% (show up more than 1 year before)…*nearly all (99%? lol)

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24
Q

If there is ocular involvment in pemphigus, what does it present as?

A

bilateral conjuntivitis *Unlike pemphigoid, no scarring or symblepharon formation

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25
Q

What is the term for the induction of a bulla with firm lateral pressure on normal-appearing skin in a patient with pemphigus vulgaris?

A

a positive nikolsky sign

26
Q

H&E and DIF buzzwords for pemphigus vulgaris (3)

A
  1. “row of tombstones” on H&E 2. Tzanck cells 3.”chicken wire” pattern on DIF
27
Q

What 4 entities have Tzanck cells in their histology?

A
  1. Pemphigus vulgaris 2. HSV 3. Warty dysker/darier dx 4. VZV
28
Q

What three markers can be demonstrated in the intercellular spaces between the epithelial cells on direct immunofluorescence of Pemphigus vulgaris?

A

IgG, IgM, or C3

29
Q

Since pemphigus is commonly treated with systemic corticosteriods, what are 7 side effects of their long term use?

A
  1. Diabetes mellitus 2.Adrenal suppression 3. Weight gain 4. osetoporosis 5.Peptic ulcers 6.severe mood swings 7. susceptible to infections
30
Q

What is the current mortality rate of Pemphigus vulgaris?

A

5-10% (complications of systemic corticosteriod)…comparred to 60-90% before systemic corticosteriods (infections and electrolyte imbalances)

31
Q

What are 2 alternate names for paraneoplastic pemphigus?

A

1.Neoplasia-induced pemphigus 2.Paraneoplastic autoimmune multiorgan syndrome

32
Q

What are the two main hematologic neoplasms that are associated with paraneoplastic pemphigus? What is a benign hematologic neoplasm?

A

1.lymphoma 2.chronic lymphocytic leukemia…b9: Castleman Dx (angiofollicular lymph node hyperplasia)

33
Q

What cytokine is the presumed cause of paraneoplastic pemphigus?

A

abnormal levels of IL-6 (produced by patient’s lymphocytes in response to the tumor)

34
Q

What fraction of paraneoplastic pemphigus cases develop before the discovery of a neoplasm?

A

1/3

35
Q

Which clinical feature of paraneoplastic pemphigus helps distinguish it from pemphigus vulgaris?

A

Palmer and plantar bullae (+in PP and - in PV)

36
Q

Crusting of the lips in paraneoplastic pemphigus is identical to what condition?

A

Erythema multiforme

37
Q

70% of paraneoplastic pemphigus patients have cicatrizing conjunctivis, like what other condition?

A

cicatricial pemphigoid

38
Q

What is the term for a paraneoplastic pemphigus patient who has the lining of the bronchiolar mucosa slough and occlude the bronchiolar lumina?

A

Bronchiolitis obliterans

39
Q

While DIF of paraneoplastic pemphigus has a lot of crossover with PV, what is one specific family of desmosomal components affected in PP? Which 2 specifically?

A

antibodies against the PLAKIN family of desmosomal components … 1.PeriPlakin and 2. Envoplakin

40
Q

What is the mortalitiy rate for paraneoplastic pemphigus?

A

90%

41
Q

What are the 2 alternate names for Mucous Membrane Pemphigoid??

A

1.Cicatricial pemphigoid 2.Benign mucous membrane pemphigoid

42
Q

Which is more common? MMP or PV?

A

MMP is 2x more common than PV

43
Q

MMPs alternate name of ‘cicatricial’ is in reference to which aspect of this condition?

A

the cicatrix formation of the conjunctiva (the oral lesions do not tend to scar)

44
Q

MMP: average age range at dx? sex predilection?

A

50-60 years old…2:1 female predilection

45
Q

What is the term for the adhesion between the bulbar and palpebral conjunctivae in MMP? What are two other terms that can occur to the eye?

A

Symblepharon…1)entropion (upper eyelid inward) 2)trichiasis (eyelashes rub against the eye)

46
Q

What % of patients with oral lesions in MMP also develop ocular lesions? What is the earliest change?

A

25%…subconjunctivial fibrosis (seen in slit lamp exam)

47
Q

What is the term for vaginal lesions in a female pt with MMP?

A

Dyspareunia

48
Q

What are the 4 immune deposits found in DIF for MMP?

What combination might be more severe?

A
  1. IgG 2.C3, although 3.IgA and 4.IgM may also be

identified. …when IgG and IgA deposits are found in the same patient, the disease may be more severe

49
Q

What is the DIF pattern for MMP?

A

a continuous linear band of immunoreactants (IgG, C3, IgA, and IgM) at the basement membrane zone

50
Q

INDIRECT immunofluroescence of MMP has shown production of what two proteins?

A

1) epiligrin (laminin-5) (a component of the basement membrane) and 2) α6 integrin (a component of the hemidesmosome)

51
Q

What are the 3 mouth-full entities that can mimic MMP?

A

1.Linear IgA bullous dermatosis 2.Angina bullosa hemorrhagica 3.Epidermolysis bullosa acquisita

52
Q

Linear IgA bullous dermatosis shows what pattern on DIF? How is it distinguished from MMP clinically?

A

characterized by the linear deposition of only IgA along the basement membrane zone…. LIBD predominantly affects the skin

53
Q

Name that MMP mimic: a rare, poorly characterized
oral mucosal disorder that exhibits variably painful,
blood-filled vesicles or bullae, usually affecting the soft
palate of middle-aged or older adults

A

Angina bullosa hemorrhagica

54
Q

Histo for Angina bullosa hemorrhagica

A

subepithelial cleft

55
Q

WHat are 2 possible causes of Angina bullosa hemorrhagica?

A

trauma or corticosteroid use

56
Q

Which MMP mimic is an immunologically

mediated condition characterized by autoantibodies directed against type VII collagen?

A

Epidermolysis bullosa acquisita

57
Q

Epidermolysis bullosa acquisita clinically resembles the inherited dystrophic epidermolysis bullosa, how are they distinguished?

A

epidermolysis bullosa acquisita typically affects middle-aged or older adults (thus acquired and not inherited)

58
Q

What % of Epidermolysis bullosa acquisita patients have oral lesions? (Usually assoc with cutantous lesions)

A

50%

59
Q

What is the unique diagnostic technique used for Epidermolysis bullosa acquisita?

A

Incubate in saline to create artificial separation, then DIF shows IgG on the connective tissue side where the type VII collagen resides (vs MMP where the autoantibodies are on the epithelial side / “roof” of the blister)

60
Q

What are 2 good alternatives to systemic corticosteriods if topicals are not working for MMP?

A

DAPSONE (sulfa drug derivative) or tetracycline

Neville and Damm (72 decks)

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