vesiculobullous disorders Flashcards
+ Nikolsky sign
+ asboe hansen
- FLACCID vesicles/bullae, fragile blisters, painful erosions/crusts on scalp, upper trunk and groin
- autoauntibodies for Desmoglein 1 and 3
What is it?
How to DX
How to TX
Pemphigus Vulgaris
ElISA lab with elevated des 1 & 3
H&E w/ DIF
TX:
systemic glucocortico 1-1.5mg/kg/day
rituximab (1st line)
Severe mucocutaneous erosions, dusky targetoid, rapidly progressing
What do you do?
How to dx?
treatment?
H&E w/ DIF will show ifF epidermal of intracellular spaces and basement membrane
ID malignancy and treat ASAP, requires aggressive treatment.
Erythemamultiforme
autoantibodies BP 230 &180
Bullous Pemphigoid
subepidermal blister of mucose (oral most common)
Early prodromal phase is nonbullous and very pruritic
Urticarial plaques, patches, and erythema w/ tense large bullae
- Nikolsky
bilateral and symmetric on flexural surfaces of extremities and trunk.
What is it?
How to TX?
Bullous Pemphigoid
H&E w/ DIF showing linear IGG and C3, salt split skin
ELISA- elevated bp 230 & 180
initially- intermediate doses of prednisone
dapsone, mycophenolate mofetil, azathioprine, methotrexate, tetracycline, niacinamide, rituximab
Gold standard for diagnosing blistering disorder
DIF
where is DIF performed
perilesoinal skin, transported in michel’s medium
What should one consider when prescribing oral steroids for > than 12 weeks?
osteoporosis
peptic ulcer prevention
Intensely pruritic
mostly excoriations, urticairal small papulovesibular lesions that are clustered
common on extensor surfaces, symmetrical
What is it ?
How to DX?
How to TX?
H&E, dif shows granular deposition of IgA on dermal papillae
Dermatitis Herpetiformis
life long fluten free diet control; eliminating grains, allowing rice oats and corn
1st line drug: dapsone 25-150mg daily start low, increase slow
2nd line sulfapyridine
What to look for when managing a patient on dapsone
Hemolytic anemia (all patients)
methemoglobinemia
hypersensitivity reaction
agranulocytosis
peripheral neuropathy
symmetrical, favors extensor surfaces
pruritic annualr, arcuate, string of pearls or cluster or jewels
Secondary crusted excoriations
CBDC- rings of grouped bullae usually around genitalia, face and perioral
How to DX?
What is it?
How to treat?
H&E w/ DIF revealing linear deposition of IgA
Linear IGA
1st line; dapsone
2nd line prednisone, sulfapyridine, cochicine
Acquired blistering disorder resulting from trauma
very common on hands/feet/fingers/toes, fragility leading to poor quality of life
heals with milia/scarring
how to DX?
what is it?
How to treat?
h&e revealing subepidermal blister with neutrophillic infiltrate
DIF- linear deposition of IgG at BMZ
EPIDERMOLYSIS BULLOSA ACQUISITA
DAPSONE, COLCICHINE
PREDNISONE
educate on avoiding trauma or friction
inherited, presents soon after birth
mechanobullous skin and mucosa fragility
refer to genetic counselor
epidermolysis bullosa
rare blistering disorder of pregnancy, urticarial papules, annular or polycyclic plaques vesicles/bullae, 2nd and 3rd trimester, involves the umbilicus, associated with GRAVES, recurs with subsequent pregnancy, resolves 6 months post delivery
Autoantibodies bp 180 & 230 and collagen XVII
How to DX?
How to treat?
What is it ?
Pemphigusgestationis
H&E-subepidermal blister, DIF- linear deposition of c3 along BMZ
ELISA increased 180
topical steroids, antihistamines oral steroids if severe
