Matthys: Vesiculobullous Diseases Flashcards
Vesicle
-a blitster less than 1 cm in size
Bulla
bilster greater than 1 cm
Vesiculobullous
-blisters between > and < 1 cm
Impetigo
- highly contagious gram + bacterial infection of the superficial layers of the skin
- Staph Aureus group 2 phage 71?GABHS
- Bullous/nonbullous
Path of impetigo
- Group 2 S. Aureus
- nares small percentage groin, hand, rectum, pharynx
- normal flora
- exfoliative endotoxins A nd B
- neonates and infants most common for bullous
Clinical presentation of impetigo
- rapid blister, asymptomatic
- hot weather
- crowded condition
- poor immune function
- atopic dermatitis, herpe infection, trauma
- flaccid <1 cm bulla, usuallly ruptured
- collarete of scale
- no sore throat or LAD
- face most common ,but anywhere on body
ddx for impetigo
- Herpes simplex
- tinea pedis
- bullous pemphigoid
- staph scalded skin
- pemphigus vulgaris
Tx for impetigo
-abx
Staphylococcal scalded skin syndrome
- toxin mediated exfoliative dermatitis
- severe impetigo to widespread skin denudation
- exfoliation followed by painful erythema
- infants and young children
- nurseries and daycare
pathophys of scalded skin syndrome
- S aureus type 2
- exotoxine 1 and 2
- blister at the granular layer of the epidermis splitting the desmosomes
clinical presentation of scalded skin sydnrome
- focus of infection
- fever, faint orange red macules
- nikolsky sign
- paper like wrinklying of the peidermis
- cultures are sterile
- dramatic….. heals in 5-7 days
what is nikolsky sign?
-dislodgement of intact superficial epidermis by a shearing force, indicating a plane of cleavage in the skin
ddx for scalded skin syndrome
- scarlet fever
- kawasaki disease
- TEN
- EM
- child abuse
tx of scalded skin syndrome
- abx
- fluids
- make the diagnosis
- culture/bx….. frozen section
Pemphigus vulgaris
- Autoimmune blistering disease of the skin and oral mucosa
- circulating IgG antibody against the intraepidermal keratinocytes
pathophys of pemphigu vulgaris
- Binding of IgG autoantibodies causes loss of adhesion between keratinocytes
- Desmoglein 3
- circulating IgG1 and 4
- disease activity correlates with circulating antibodies
clinical presentation of pemphigus vulgaris
- all races
- 50-70% oral mucosa involvemment
- flaccid bulla on the skin with an erosion in the oral mucosa
- +nikolsky sign
- +absoe-Hansen sign- lateral pressure on the bulla will spread the bulla to uninvolved skin
Causes of pemphigus vulgaris
- genetic
- age
- disease association…. myasthenia gravis and thymoma
ddx of pemphigu vulgaris
- Bullou spemphigoid
- Hailey-Hailey disease
- linear IgA disease
- Pemphigus Foliaceus
dx and tx of pemphigus vulgaris
- biopsy
- IDIF
- Prednisone, Imuran
- Ophthamologist
Herpes Simplex
- 1 is above belt, 2 is below
- 1 causes encephalitis 60-80% mortality
- Primary infection, latency, and recurrence
pathophys of HSV
- 1= respirator droplets
- 2= genital contact
- cytolytic infection with fluid filled vesicles
- 70-90% of population has HSV1 vs 22% has HSV2
clinical presentation of HSV
- asymptomatic to painful; prodrome of itching and burning
- herpetic whitlow andherpes gladiatorum
- recurrent lesions common
- immune status
- encephalitis
- Vesicular or ulcerative on an erythematous base
- oral, genital, body, keratoconjunctival, encephalitis, anal
- Seizures, headache
ddx of HSV
- Chancroid
- Urethritis
- HAnd foot and mouth disease
- Proctitis
dx and tx of hsv
- culture or biopsy (tzanck)
- antivirials
Chicken pox
-self limiting, contagious, viral infection
-4 million children per year
-4000 million in lost wages per year
=- 50-100 deaths annually in US
pathophys of chicken pox
- primary varicella virus; herpes family type 3
- respirator and direct contact with skin
- 10-21 day incubation
- Infectious 1-2 days prior to the rash and until the lesions crust
clinical presentation of chicken pox
- hx of recent outbreaks
- itchy rash
- “oval teardrop on an erythematous base”
- “dew drop on a rose pedal”
- face and trunk
- All stages
- fever, shallow aphthous ulcers on oral mucosa
- spares the distal extremity
ddx of chicken pox
- impetigo
- herpes simplex
- herpes zoster
dx and tx of chicken pox
- no ASA
- VZIG
- Antivirals
- Antihistamines
Bullous pemphigoid
- chronic, AI, subepidermal blistering disease that RARELY involves the oral mucosa
- Autoantibodies of IgG against the BP antigens 230 and 180 (BP Ag1 and 2)
- uncommon
Pathophys of Bullous pemphigoid
- IgG bind to skin atibodies activation complement and inflammatory mediators
- Inflammatory cells release proteases causeing bulla formation
- Serum levels of BP Ag2 may correlate with disease activities in some pts
clinical presentation of Bullou spemphigoid
- subactute or acute formation of tense bulla
- UV radiation or drug induced
- Bullous, vesicular, urticarial , acral in children
- Causes include genetic, age, epitope spreading
ddx for bullous pemphigoid
- cicatrical pemphigoid
- drug induced pemphigoid
- Herpes Gestationis
dx ant x of bullous pemphigoid
- Biopsy
- IDIF (indirect immunofluoresence?)
- Prednisone and other immunosuppresives
Polymorphous light eruption
- idiopathic photodermatoses relating to sun exposure
- papules, vesicles, and plaques
- 10-21% of all people
- 75% American Indian
- happens more with women
pathophys of PLE
- unknown
- type IV delayed hypersensitivity rxn
- UV-A induced ICAM-1
Clinical presentation of PLE
- sunlight is etiologic factor most of time
- spring, lessens throughout summer
- about 30 minutes after sun exposure
- papules, vesicles, urticaria, or EM type features
- UV-A or UV-B
ddx for PLE
- subacute lupus
- contact dermatitis
- solar urticaria
dx and tx for PLE
- R/O porphyria, lupus
- photopatch testing
- photo testing MED
- Topical steroid, oral or IM steroids, antimalarials
Dermatitis Herpetiformis
- Immune mediated skin disease with a gluten sensitive enteropathy
- rare in african american
pathophys of DH
- skin and GI affected
- circulating immune complexes are found
- IgA binds antigen in bowel and skin
- Increased expression of HLA-B1, B8, DR8, and DRQ
- Patient have uniform gluten sensitivity
clinical presentation of DH
- pruritic, burning skin blisters distributed in a symmetric fashion; grouped
- Lasts days to weeks
- Urticarial wheal to vesicle to bulla
ddx for DH
- Allergic contact dermatitis
- Atopic Dermatitis
- Scabies
- Neurotic excoriations
- Papular Urticaria
- Bullous pemphigoid
dx and tx for DH
- HE and DIF biopsy
- Clinical exam
- Be aware of associated diseases
- Bapsone and gluten free diet
Erythema Multiforme
- Benign self limited eruption classically seen as targetoid or iris shaped macules or vesiculopapules on the palms or soles
- Minor/major
- 50% under age 20
- seems relatively rare
pathophys of EM
- not understood/HSV cell mediated response
- Infection major cause
- Drugs….. sulfa/phenytoin/ PCN/allopurinol
- Foods, sunlight, contactants, etc
clinical presentation of EM
- dull red macules or papules with vesicles on palms or soles with little mucosal involvement
- Iris or target like
- bilateral and often symmetric
- polycyclic or arcuate
- Koebner Phenomenon
where will EM present on the body
- mucosal involvement 70%
- lips
- anus
- eyes
- oropharynx
ddx for EM
- drugs
- syphillis
- psoriasis
- BP
- SSSS
- Kawasaki’s disease
dx and tx for EM
- biopsy
- find cause
- antivirals
- Steroids?
Toxic Epidermal necrolysis
- extensive full thickness skin detachment
- EM spectrum
- Early sx non specific
Pathophysiology of TEN
- HLA B12
- SLE
- HIV
- Medicines, infection: cell mediated cytotoxic rxn against epidermal cells, CD8 cells, macs, cytokines
clinical presentation of TEN
- 1-3 week proderome
- fever, skin tenderness
- skin pain, burning
- Newly added drugs most common
Clinical presentation of TEN
- Morbilliform, EM type eruption
- Skin with crinkled surface
- sheet like epidermal loss
- +nikolsky sign
- Mucosal membrane involvement
ddx for TEN
- EM
- Scarlet fever
- GVHD
- SSSS
- Exfoliative dermatitis
- Genreralized bullous fixed drug eruption
dx and tx for TEN
- D/C drug suspected
- IV fluids
- Burn unit
- Steroids as a last resort
- IV Ig
- Prevent infection
