Chapter 1 - Vesiculobullous Diseases Flashcards

1
Q

What are the vesiculobullous diseases?

A
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2
Q

What are the vesiculobullous ulcerative lesions (based on etiology)?

A
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3
Q

What are clinical features of primary herpes simplex?

A

Few primary infections result in clinical disease (silent)

Oral and perioral vesicles rupture, forming ulcers

Intraoral lesions may be found on any surface

Systemic signs: fever, malaise, cervical lympadenopathy

Self-limited disorder (immunocompromised patients have more severe disease)

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4
Q

What are the treatment options for primary herpes simplex?

A

Acyclovir and analogs may control the virus if caught within 72 hours of onset. Treatment must be provided early to be effective.

TLC

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5
Q

What is the etiology of secondary herpes simplex? What are triggers? Prodromal symptoms?

A

HSV-1

Triggers: sunlight, stress, immunosuppression, routine dental caare, trauma

Prodromal: tingling, burning, or pain

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6
Q

What are the clinical features of Secondary Herpes Simplex?

A

Affects perioral skin, lips, gingiva, and palate

Generalized vesicles or ulcers

Tingling, swollen erythematous and bleeding gums

Self-limited

Gingiva and hard palate (Keratinized mucosa)

Bilateral presentation

*Note: can always present on tongue as white papules with minimal oral pain

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7
Q

Primary HSV Differential Diagnosis

A
  • Streptococcal Pharyngitis
  • Erythema Multiforme/Stevens Johnson syndrome
  • Coxsackie virus infection
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8
Q

Secondary HSV Differential Diagnosis

A
  • Aphthous ulcers (these occur on non-keratinized tissue)
  • Herpes zoster (both have Tzanck cells with glassy nuclear inclusions but zoster is unilateral and much more painful)
  • Impetigo
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9
Q

Differentiate erythema multiforme from herpes simplex.

A

Erythema multiforme: spares the gingiva; Type 3 hypersensitivity; target/bullseye lesions; can be a viral etiology (HSV) or drug-induced (penicillin); usually without a vesicular stage

Herpes simplex: smaller lesions; vesicle stage; viral etiology

Both are acute diseases and self-limited

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10
Q

Primary Varicella Zoster Infection

A

Also known as Chicken Pox; DNA virus

  • Droplet infection usually in children
  • Crops of vesicular eruptions on trunk, head and neck with pruritis
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11
Q

What are complications of Primary Varicella Zoster Infection?

A

Pneumonitis

Encephalitis

Inflammation of other organs

Fetal abnormalities if infection occurs during pregnancy

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12
Q

Clinical features of Secondary Varicella Zoster Infection

A

Also known as Shingles; only seen in patients who have been previously exposed to chicken pox

-Unilateral maculo-papular lesions

Follows dermatome paterns of the trunk, head and neck

Severe pain

Prodromal syndrome: tingling, etc. prior to symptoms/lesions showing up

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13
Q

Ramsay Hunt Syndrome

A

Complication of Varicella Zoster (?)

Severe unilateral facial palsy with vesicle development occuring with onset of facial weakness on tongue, hard palate or ear

CN VIII: Vestibulocochlear

Other symptos: hearing loss, tinnitus, vertigo, glossopharyngeal/vagal symptoms (trouble swallowing)

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14
Q

Treatment options for Varicella Zoster Infection

A

Acyclovir 800mg 5x/day for 7-10 days

GABA Pentine (steroid alternative, which are contraindicated in immunocompromised pt)

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15
Q

What are some of the complications of Secondary Varicella Zoster?

A

Infection of ulcers, post-herpetic neuralgia, motor paralysis, and ocular inflammation

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16
Q

Varicella Zoster Differential Diagnosis

A

HSV

Hand Foot and Mouth disease

17
Q

Hand-foot-mouth Disease

A

Picornovirus

Airborne or fecal-oral contamination

Children under 5 years old (daycare)

Symptoms: low-grade fever, lymphadenopathy, sore mouth, and lesions on hands, feet, and in the mouth

Areas most commonly affected: Palate, Tongue, and buccal mucosa

18
Q

Herpangina

A

Coxsackie type-A virus

Feco-oral transmission and most common in children

Bilateral presentation limited to posterior oral cavity

Systemic symptoms: fever, malaise, dysphagia, sore throat

Summer to early Fall

19
Q

Measles/Rubeola

A

Paramyxovirus

Airborne droplets (like varicella)

Most common in children in winter time

Koplik’s spot in buccal mucosa

-Erythematous macule with necrotic center followed by skin rash

20
Q

What is the histopathology of Measles/Rubeola?

A

Warthin-Finkeldey giant cells (multinucleated macrophages seen in lympoid tissues)

21
Q

Measles/Rubeola Differential Diagnosis

A

Chicken pox (primary varicella-zoster)

22
Q

German Measles (Rubella)

A

Togavirus

Clinical features of measles but mild and short-lived (fever, respiratory symptoms, and rash)

No Koplik’s spots

23
Q

What layers do pemphigus foliaceus/erythematous affect?

A

Upper prickle cell/spinous layer

*Note Pemphigus vulgaris affects the entire epithelium and involves the oral mucosa

24
Q

Clinical features of Pemphigus vulgaris

A

Occurs most often in the 4th and 5th decade

  • Associated with other autoimmune disorders (myasthenia gravis/lupus erythematosus/RA/Hashimoto thyroiditis/Sjogren’s)
  • Vesiculob__ullous with oral lesions commonly preceding cutaneous lesions (up to 1 year), then rupturing to form ulcers

Positive Nikolsky’s sign: gentle traction on unaffected mucosa results in vesicular eruption

25
Q

Pemphigus vulgaris Differential Diagnosis

A
  • Mucous membrane pemphigoid
  • Erythema multiforme
  • Aphthous ulcers
  • Paraneoplastic pemphigus
  • Erosive lichen planus
26
Q

Treatment options for Pemphigus vulgaris

A

Systemic corticosteroids (side-effects)

Anti-CD-20 monoclonal therapy (Rituximab)

27
Q

What are some of the side effects of topical and systemic corticosteroids?

A
28
Q

Histopathology of Pemphigus vulgaris

A

Agent: IgG/IgA/C3

Target: Desmoglein-3

Indirect IF assay and Direct IF assay

29
Q

Mucous Membrane Pemphigoid

A

Affects oral and ocular mucous membranes

Antigenic targets: Laminin 5

Low circulating antiboies

Greater occurence in women

Presentation: bright red patch or superficial ulcers in the attached gingiva

Rarely bullae as they are short lived

Positive Nikolsky’s sign

30
Q

What are symblepharon, entropion, and trichiasis?

A

Symblepharon: scar at the canthus

Entropion: inversion of the eyelashes

Trichiasis: trauma to cornea

*All associated with MMP (mucous membrane pemphigoid)

31
Q

Differential Diagnosis of Mucous Membrane Pemphigoid (MMP)

A

-Pemphigus vulgaris

*Erythematous lichen planus

  • Linear IgA disease
  • Discoid lupus erythematosus

*Lichenoid mucositis

Can’t use indirect IF assay due to low circ. ABs

*=more common and important to understand/differentiate between

32
Q

Histopathology of MMP

A
  • Sub-epithelial clefting (basal layer lifts from basement membrane)
  • Hemidesmosomal juntion: lesions last longer/heal slower than Pemphigus vulgaris because full thickness of epithelium is lifted/sloughed off
33
Q

Bullous Pemphigoid

A

Closely related to MMP: different antigens rsponsible: BP230 and BP180 (vs. BP180 and laminin-5 for MMP)

  • Lesions on skin and oral mucosa
  • Common in elderly (7th/8th decade)
34
Q

Epidermolysis Bullosa

A

Characterized by _blisters (bulla) at sites of minor trauma (_also due to stress)

  • Acquired form: IgG (sub-BMZ) and Type 7 collagen ABs below Lamina densa
  • Hereditary form: no circulating ABs; oral lesions common: heal with scarring