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Fitz 9th > 52 - Pemphigus > Flashcards

52 - Pemphigus Flashcards

1
Q

4 types of pemphigus

A

Vulgaris
Foliaceus
Paraneoplastic
IgA pemphigus

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2
Q

Localized forms of pemphigus vulgaris

A

Pemphigus vegetans of Hallopeau > Pemphigus vegetans of Newman (more extensive)

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3
Q

Localized form of pemphigus foliaceus

A

Pemphigus erythematosus

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4
Q

More common in Jews and probably in people of Mediterranean descent and from the Middle East

A

Pemphigus vulgaris

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5
Q

Endemic foci of pemphigus foliaceus in Brazil, Colombia, and Tunisia

A

Fogo selvagem

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6
Q

Fogo selvagem vs pemphigus foliaceus

A

Fogo selvagem - children and young adults; anyone in an endemic area may be susceptible
Sporadic PF - middle- aged and older patients; in genetically related family members

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7
Q

Positive Nikolsky sign differentials

A

Pemphigus vulgaris
SSSS
SJS-TEN

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8
Q

Pemphigus vegetans sites of predilection

A

Intertriginous areas
Scalp
Face

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9
Q

Pemphigus vegetans prognosis

A

Milder disease and higher chance of remission

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10
Q

Mucous membranes most often affected by PV

A

Orophryngeal cavity

Nasal mucosa

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11
Q

In the majority of patients, _____ are the presenting sign of PV

A

Painful mucous membrane erosions

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12
Q

Features of a pemphigus-lupus erythematosus overlap

A

Senear-Usher syndrome

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13
Q

Drug-induced pemphigus caused by

A

Penicillamine

Captopril

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14
Q

Thiol-containing foods such as _____ may precipitate PV

A

Garlic
Leek
Onion

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15
Q

Diseases associated with PV and PF

A

Myasthenia gravis

Thymoma

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16
Q

Pemphigus autoantigens

A

Desmogleins

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17
Q

PF antigen

A

Desmoglein 1

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18
Q

PV antigen

A

Desmoglein 3

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19
Q

Antibodies in mucosal-dominant PV

A

Anti-desmoglein 3 antibodies

20
Q

Neonatal pemphigus resolves by approximately

A

6 months - concurrent with the disappearance of maternal IgG from the circulation

21
Q

Constant region of the antibody is required for blister formation in _____. In _____, the variable region of the antibody is sufficient

A

Pemphigoid and EBA

Pemphigus

22
Q

Pathogenic ang predominant IgG subclass in PV and PF

A

IgG4

23
Q

Pathogenic PV and PF autoantibodies bind calcium-sensitive conformational epitopes in the _____, whereas nonpathogenic antibodies tend to bind more _____

A

Amino terminal extracellular domains of desmogleins

Membrane proximal extracellular domains

24
Q

Hypothesis which proposes that pathogenic antibodies directly interfere with desmoglein adhesive interactions, causing acantholysis

A

Steric hindrance

25
Q

Autoantibodies against one desmoglein isoform inactivate only that isoform and that another isoform co-expressed in the same area can compensate in adhesion

A

Desmoglein compensation

26
Q

The depletion of desmogleins by pemphigus antibodies may lead to loss of desmogleins resulting in smaller desmosomes and/or their defective function in adhesion, a scenario referred to as

A

Dsg nonassembly depletion hypothesis

27
Q

Among Ashkenazi Jews with PV, the serologically defined HLA-_____ is predominant, whereas in other ethnic groups with PV, the _____ allele is more common

A

DR4

DQ1

28
Q

DIF sample

A

Perilesional skin

29
Q

IIF sample

A

Serum

30
Q

ELISA sample

A

Serum

31
Q

Hallmark of pemphigus is the finding of _____ autoantibodies against the cell surface of keratinocytes

A

IgG

32
Q

DIF finding in pemphigus erythematosus

A

IgG and C3 in the basement membrane zone in addition to the epidermal cell surface IgG

33
Q

Can remain positive for weeks to months after healing of the skin lesions, making it a good diagnostic test if a patient should present with no active lesions

A

IIF

34
Q

Substrate that is more sensitive for detecting PV antibodies

A

Monkey esophagus

35
Q

Superior substrate for detecting PF antibodies

A

Guinea pig esophagus or normal skin

36
Q

Test that can differentiate PV and PF

A

ELISA

37
Q

Often the cause of death in PV and PF

A

Infection

38
Q

Initiation of treatment for PV vs PF

A

PV - should be treated at its onset, because it will ultimately generalize and the prognosis without therapy is very poor
PF - may be localized for years; do not necessarily require treat with systemic therapy - use of topical corticosteroids may suffice

39
Q

Mainstay of therapy for pemphigus

A

Systemic administration of glucocorticoids

40
Q

Full systemic dose of glucocorticoids for pemphigus

A

1.5 mg/kg/d of prednisone equivalent for 2-3 weeks

41
Q

Monoclonal anti-CD20 antibody approved by the FDA for therapy of pemphigus

A

Rituximab

42
Q

Immunosuppressive drugs that may offer protection against Pneumocystis carinii infection

A

MMF

Dapsone

43
Q

Side effects of IVIg

A

Stroke
Deep venous thrombosis
Aseptic meningitis

44
Q

Treatment for pemphigus that does not increase the risk of infection

A

IVIg

45
Q

Side effects of cyclophosphamide

A

Hematuria
Infection
Transitional cell carcinoma of the bladder
Infertility

Fitz 9th (108 decks)

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