Dz of epidermis, dermis and BMZ (and subQ) Flashcards
Rare, acquired AI blistering disease of the epidermis
Pemphigus vulgaris
pemphigus vulgaris is d/t…
auto-ab against desmoglein 1&3
a positive Nikolsky’s sign is found in which disease?
pemphigus vulgaris
outcome and treatment of pemphigus vulgaris
fatal if not treated
immunosuppression - prednisone, azathioprine, mycophenoate mofetil, Rituximab
Common AD disease with mut in profilaggrin gene
Ichthyosis vulgaris
filaggrin is essential for….
cornified envelope
disease that predisposes or is associated with allergic conditions
ichthyosis vulgaris
Acquired AI dz of the dermis characterized by localized sclerosis
Morphea/localized scleroderma
what age range and gender are most commonly affected by morphea?
children and adult F
dz that presents as erythematous and indurated plauqes that slowly expand, leaving behind fibrotic or atrophic scars
morphea
what complications can be associated with morphea? which are not?
joint and neurologic complications
- not internal organ involvement
Acquired AI dz of the dermis, most common in middle-aged F
Systemic sclerosis
limited systemic sclerosis is AKA CREST. what does CREST stand for?
calcificus cutis Reynaud's Esophageal dysmotility Sclerodactyly Telangiecatasia
Dermal disease that includes pulmonary fibrosis, renal failure, GI dz, Cardiac dz
diffuse systemic sclerosis - widespread sclerosis of the skin
Acquired disorder of the SubQ fat, presenting with tender red nodules on shins
Erythema nodosum
erythema nodosum is most common in…
young females
panniculitis
inflammatory process involving the fat
most common cause of erythema nodosum
infections, UR strep
- also, medications, sarcoidosis, IBD, malignancy
outcome of erythema nodosum?
nodules on shins arise in crops, then slowly involute over the course of a few days/weeks
which type of collagen is in the lamina densa?
type IV
which type of collagen is in the sublamina densa?
type VII
list 3 molecules collagen type VII binds to
collagens types I and IV, laminin 332/5
most common AI bullous dermatosis
bullous pemphigoid
describe the stages of bullous pemphigoid
- starts with pleomorphic clinical features, e.g. highly pruritic urticaria w/o blistering
- next, tense blisters
bullous pemphigoid is d/t
auto-Ab against BP 1 and 2
What does IIF show for Bullous Pemphigoid?
epidermal staining
which as the better prognosis, bullous pemphigoid or pemphigus vulgaris?
BP
what age range do bullous pemphigoid and pemphigoid vulgaris show up in?
elderly
elderly pt presents with an AI dz with recurrent blistering of mucous membranes (eyes, mouth, etc.) and occasionally the skin
mucous membrane pemphigoid
sequelae of MMP include…
blindness (20%), scars, strictures, synechiae (iris adheres to the cornea)
MMP is d/t…
auto-ab to BP 180, or laminin 332/5
MMP subtype with anti-laminin 332-Ab has an association with__________, so the doctor should ___________
malignancies - 30% of cases, so perform appropriate CA screening
in a pt with MMP, IIF will stain on the….
epidermis - or dermis if anti laminin 332
rare acquired AI bullous dermatosis
epidermolysis bullosa acquisita
presentation of EB-acquisita
blistering and erosions in response to slight trauma - prevalent on mechanically stressed skin (hands, feet, elbows, knees)
- often involves the oral mucosa (hemorrhagic errosions)
EB-acquisita is d/t…
anti-collagen type VII-Ab
IIF stains where for EB-acquisita
dermal side
Inherited EB is d/t
mutations in genes encoding BMZ proteins
group of inherited skin fragility disorders
epidermolysis bullosa
AD-inherited dz of the BMZ targeting keratins 5 and 14
EB-simplex
AR-inherited dz of the BMZ targeting laminin 332 and BP180
Herlitz Junctional EB
AD and AR-inherited dz of the BMZ targeting collagen type VII
Dystrophic EB
subtype of EB d/t mutation in kindling 1
kindler syndrome
keratohyalin granules contain
filaggrin
which layer of the epidermis is keratin synthesized in
stratum spinosum
lamellar granules contain
hydrolytic enzymes, ceramides, cholesterol and fatty acids
what protein is responsible for thef protein cornified envelope
filaggrin
what is the most abundant type of collagen in the skin
type I
which corpuscles are for light touch
meissner’s
which BP is cytoplasmic and belongs to the plakin family
BP 230
what is BP 180 AKA?
collagen type XVII
lamina densa contains primarily which two molecules?
collagen IV and laminin 332
