Chapter 1 - Vesiculobullous Diseases Flashcards
What are the vesiculobullous diseases?
What are the vesiculobullous ulcerative lesions (based on etiology)?
What are clinical features of primary herpes simplex?
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)
What are the treatment options for primary herpes simplex?
Acyclovir and analogs may control the virus if caught within 72 hours of onset. Treatment must be provided early to be effective.
TLC
What is the etiology of secondary herpes simplex? What are triggers? Prodromal symptoms?
HSV-1
Triggers: sunlight, stress, immunosuppression, routine dental caare, trauma
Prodromal: tingling, burning, or pain
What are the clinical features of Secondary Herpes Simplex?
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
Primary HSV Differential Diagnosis
- Streptococcal Pharyngitis
- Erythema Multiforme/Stevens Johnson syndrome
- Coxsackie virus infection
Secondary HSV Differential Diagnosis
- 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
Differentiate erythema multiforme from herpes simplex.
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
Primary Varicella Zoster Infection
Also known as Chicken Pox; DNA virus
- Droplet infection usually in children
- Crops of vesicular eruptions on trunk, head and neck with pruritis
What are complications of Primary Varicella Zoster Infection?
Pneumonitis
Encephalitis
Inflammation of other organs
Fetal abnormalities if infection occurs during pregnancy
Clinical features of Secondary Varicella Zoster Infection
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
Ramsay Hunt Syndrome
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)
Treatment options for Varicella Zoster Infection
Acyclovir 800mg 5x/day for 7-10 days
GABA Pentine (steroid alternative, which are contraindicated in immunocompromised pt)
What are some of the complications of Secondary Varicella Zoster?
Infection of ulcers, post-herpetic neuralgia, motor paralysis, and ocular inflammation
Varicella Zoster Differential Diagnosis
HSV
Hand Foot and Mouth disease
Hand-foot-mouth Disease
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
Herpangina
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
Measles/Rubeola
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
What is the histopathology of Measles/Rubeola?
Warthin-Finkeldey giant cells (multinucleated macrophages seen in lympoid tissues)
Measles/Rubeola Differential Diagnosis
Chicken pox (primary varicella-zoster)
German Measles (Rubella)
Togavirus
Clinical features of measles but mild and short-lived (fever, respiratory symptoms, and rash)
No Koplik’s spots
What layers do pemphigus foliaceus/erythematous affect?
Upper prickle cell/spinous layer
*Note Pemphigus vulgaris affects the entire epithelium and involves the oral mucosa
Clinical features of Pemphigus vulgaris
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
Pemphigus vulgaris Differential Diagnosis
- Mucous membrane pemphigoid
- Erythema multiforme
- Aphthous ulcers
- Paraneoplastic pemphigus
- Erosive lichen planus
Treatment options for Pemphigus vulgaris
Systemic corticosteroids (side-effects)
Anti-CD-20 monoclonal therapy (Rituximab)
What are some of the side effects of topical and systemic corticosteroids?
Histopathology of Pemphigus vulgaris
Agent: IgG/IgA/C3
Target: Desmoglein-3
Indirect IF assay and Direct IF assay
Mucous Membrane Pemphigoid
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
What are symblepharon, entropion, and trichiasis?
Symblepharon: scar at the canthus
Entropion: inversion of the eyelashes
Trichiasis: trauma to cornea
*All associated with MMP (mucous membrane pemphigoid)
Differential Diagnosis of Mucous Membrane Pemphigoid (MMP)
-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
Histopathology of MMP
- 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
Bullous Pemphigoid
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)
Epidermolysis Bullosa
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
