Vesicular Bullae & Desquamation Flashcards
What age does bullous pemphigoid normally appear?
after 65 years
What is bullous pemphigoid?
A chronic, autoimmune, inflammatory, subepidermal, blistering disease
Autoantibodies attach to the skin basement membranes and activate inflammation
How long does bullous pemphigoid last?
persists from months to years, with remissions and exacerbations
it is relatively uncommon
What is the most common form of bullous pemphigoid?
generalized bullous form
What are the clinical features of bullous pemphigoid?
generalized bullous form: tense bullae arise on any part of skin surface, most commonly in flexural areas
- can occur after other chronic inflammatory conditions, such as lichen planus and psoriasis
- acute/subacute onset
- intense pruritus
- lesions may our intra-orally (NOT common)
- lesions heal without scarring
What is the “workup” for bullous pemphigoid?
- Indirect immunofluorescence
- Skin biopsy–from edge of blister + specimen for direct immunofluorescence (must be taken from normal looking peri-lesional skin, highlights deposits of antibodies)
What is the treatment for bullous pemphigoid?
- GOAL; decrease blister formation
- topical steroids
- oral steroids
- oral antibiotics (tetracyclines)
- immunosuppresives: methotrexate, dapsone, imuran
What is Erythema multiforme (EM)?
-acute, self-limited, recurring skin condition, inflammatory
-type IV hypersensitivity reaction
-resolves 2-3 weeks, can be recurrent
-symmetric erythematous skin lesions
-TARGET LESIONS-clear with erythematous rings
MAJOR AND MINOR TYPE
Where does EM occur?
extensor surfaces, palms, soles, mucous membranes
What are precipitating factors of EM?
-herpes simplex virus most common cause
(herpes associated erythema multiforme minor)
-epstein-barr virus
How is the diagnosis for EM made?
physical and history
biopsy to rule out DDx
What are the DDx of EM?
bullous pemphigoid, contact (irritant & allergic) derm, drug eruptions, SJS, TEN
What is the treatment of EM?
Treat HSV infection–suppressive dosing for recurrent flares
-most important treatment is symptomatic treatment; oral antihistamines, soothing mouthwashes
What characterizes Stevens Johnson Syndrome (SJS)?
- blisters
- epidermal detachment
- epidermal necrosis
- involves skin + mucous membranes
How much of BSA does SJS affect?
<10%
What are the causes of SJS?
- infection
- meds: NSAIDS, Anti Gout, Psychoepileptics, ABX (antibiotic)
- Malignancy
- Idiopathic (disease with unknown origin)
What are the prodromal (early) symptoms of SJS?
- productive cough
- headache
- malaise
- arthralgias
What are clinical features of SJS?
- mucocutaneous onset: mouth, esophagus, genital
- nonpruritic
- rash can being as macules–>papules, vesicles, bullae, urticarial plaques, or confluent erythema
- Center of lesions: vesicular, purpuric (red/purple discoloration), or necrotic
- Lesions can become bullous and rupture–secondary infection of skin
What are ocular symptoms of SJS?
red eye, tearing, dry eye, pain, blepharospasm, itching, grittiness, heavy eyelid, foreign body sensation, decreased vision, burn sensation, photophobia, diplopia
–painful to chew/swallow
What signs may be noted upon examination (SJS) ?
- fever (consider systemic infection)
- orthostasis
- tachycardia
- hypotension
- altered level of consciousness
- epistaxis
- conjunctivitis
- corneal ulcerations
- erosive vulvovaginitis or balanitis
- seizures
- coma
How is SJS diagnosed?
biopsy
What is the treatment for SJS?
- care in burn unit or ICU– no specific treatment; treat symptomatically
- eliminate offending drug
- fluid/electrolyte resuscitation
- wet dressings on skin
- nutritional support–parenteral nutrition
- ophthalmology consult
- pain control
- treat secondary infections
What is the prognosis for SJS?
mortality determined by extent of skin sloughing!!!
- 1-5%
- depends on age, comorbidity, & reepithelialization usually complete in 3 weeks
What are long-term complications related to ocular problems in SJS?
- photophobia
- blurred vision
- water eyes