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
What are the defining characteristics of Toxic Epidermal Necrolysis?
- widespread erythema, necrosis, bullous detachment of the epidermis and mucous membranes
- massive exfoliation–>sepsis–>death
- potentially life-threatening disorder
How much BSA is affected in TEN?
30%
What is the etiology of TEN?
- medications most common:
- antibiotics, NSAIDS, anticonvulsants
- occurs within 1-3 wks of therapy initiation and not after 8 wks
What are the clinical features of TEN?
- vital signs: hypotension secondary to hypovolemia (decreased blood volume), tachycardia
- skin lesions begin symmetrically on face & thorax then spread to entire body
- influenzalike prodrome: malaise, fever, rash, cough, arthralgia, myalgia, rhinitis, headache, anorexia, nausea, vomiting (prodrome lasts from 1 day to 3 weeks)
What area is spared in TEN?
scalp
What does the “rash” look like in TEN?
poorly defined
erythematous macular rash
purpuric (red/purple) center
What happens to the rash in TEN over a period of days?
rash comes together to form flaccid blisters and sheetlike epidermal detachment, predominately on torso and face
What are ocular manifestations of TEN?
-acute conjunctivitis, corneal erosions, ulcers
What mucosal surfaces that can be involved in TEN?
esophagus, intestinal tract, respiratory epithelium
-bronchial epithelial sloughing may result in dyspnea (shortness of breath) and hypoxemia
What precedes skin erosions in TEN?
mucous membrane erosions (90% of cases)–precede skin erosions by 1-3 days
-also pain at site of skin lesion
When does reepithelialization occur in TEN? How long do peak symptoms last?
1-3 weeks reepithelialization
2-3 days peak symptoms
What is the diagnostic study to confirm dx in TEN?
biopsy, but history and physical will determine dx
What is the “sign” that occurs when slight lateral pressure is put on the epidermal surface, resulting in epidermal surface easily separating in TEN?
Nikolsky sign
What is the treatment for TEN?
- remove offending drug
- patient should be cared for in burn unit or ICU
- fluid and electrolyte resuscitation
- temperature regulation
- monitor respiratory states (O2, intubation)
- nutritional support-parenteral feedings
- pain control
- infection precautions
- skin dressings
What are complications of TEN?
ocular, skin scarring, pigment changes, nail loss, esophageal strictures, chronic oral and genital erosions
What is the treatment for TEN?
consults, ophthalmology, plastics, IM, hospitalist
What is the prognosis for TEN?
mortality rates range from 10-70%, but AVERAGE is 25-50%
depends on extent of involvement, age, comorbid conditions, cause of death attributed to sepsis and multi organ filure
