Term
What is unique about the melanocytes' cytostructure? |
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Definition
They have perfectly round nuclei |
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Term
In addition to manufacturing hair shafts, what else are hair follicles capable of doing? |
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Definition
harbor epithelial stem cells capable of regenerating superficial epithelial skin structures |
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Term
Where are the Merkel cells and Meissner corpuscles located, respectively? |
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Definition
w/in the epithelial basal cell layer/ in the papillary dermis |
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Term
What is lichenification and why does it occur? |
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Definition
It is the diffuse thickening of the epidermis with resultingg accentuation of skin lines. It occurs from intense, chronic rubbing. |
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Term
In which disease do we see marked acanthosis? |
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Definition
lichen simplex chronicus (often due to chronic irritation) |
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Term
What is the difference between pemphigus vulgaris and bullous pemphigoid? |
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Definition
The former deals with autoantibodies to intercellular keratinocyte connections. The latter deals with autoantibodies to dermoepidermal junction antigens. |
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Term
Dyskeratosis, the abnormal premature keratinization below the stratum granulum, typically occurs as a result of what? |
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Definition
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Term
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Definition
Influx of inflammatory cells into the epidermis (spongiosis- intercellular edema of the epidermis) |
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Term
What is hyperkeratosis, and what disease features such change? |
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Definition
hyperplasia of the stratum corneum/ find this in ichthyosis |
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Term
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Definition
hyperplasia and enlargement of contiguous dermal papillae |
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Term
What's the genetic basis of oculocutaneous albinism, and what are associating presentations? |
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Definition
AR, skin malignancies risk increased |
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Term
In addition to non-nested (linear) melanocytic hyperplasia, what else is commonly found in a lentigo? |
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Definition
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Term
What are the genetic variations that are associated with nevi being neoplasms? |
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Definition
mutations in BRAF or NRAS follwed by defect in p16/INk4a which normally arrests cyclin-dependent kinases |
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Term
What the basis behind the strong dark coloration of a blue nevus? |
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Definition
The melanocytes are deep down in the dermis, so that light gets poorly refracted |
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Term
Clinically, what is a spitz nevus often confused with? |
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Definition
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Term
What are the cytologic features of Spitz nevus? |
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Definition
large, plump cells with pink-blue cytoplasm, spindle-shaped cells, pagetoid nefts and clefts around the nests |
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Term
In a halo nevus, why is there lightening of the skin around the nevus? |
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Definition
Because the infiltrating lymphocytes respond not only against nevus cells but also against surrounding normal melanocytes |
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Term
In dysplastic nevi, what is characteristic of the nevus cell nests? |
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Definition
They may be enlarged and oftne fuse or coalesce with adjacent nests/ begin to replace the normal basal cell layer along the dermoepidermal junction |
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Term
For a dysplastic nevus with cytologic atypia, what is occurring in the dermis? |
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Definition
There is linear/lamellar fibrosis (bridging fibrosis) |
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Term
In autosomal dominant dysplastic nevus syndrome, what are the two common mutations found? |
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Definition
mutated genes in CDKN2A and CDK4 |
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Term
What does radial growth refer to? |
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Definition
horizontal spread of melanoma within the epidermis and superficial dermis |
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Term
In Lentigo Maligna, how does it usually present? |
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Definition
as an indolent lesion on the fae of older men that may remain in the radial growth phase for several decades |
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Term
How do you measure the Breslow Depth of Invasion? |
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Definition
from the granular cell layer to the base of the tumor |
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Term
For gauging the prognosis of a melanoma, at what depth above and below do we measure? |
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Definition
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Term
What are the 5 levels of invasion according to Clark? |
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Definition
Intraepidermal, Papillary, Papillary-Reticular, Reticular, and Subcutaneous |
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Term
Seborrheic Keratoses in people of color may manifest as color, multiple small lesions on the face? |
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Definition
Dermatosis papulosa nigra |
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Term
What is an association with Seborrheic Keratosis that does not involve the skin? |
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Definition
paraneoplastic syndrome- Leser Trelat |
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Term
Describe the appearance of the seborrheic keratosis? |
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Definition
well-demarcated coinlike pigmented lesion containing dark keratin-filled surface plugs |
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Term
What type of cysts are associated with seborrheic keratosis? |
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Definition
invagination and horn cysts |
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Term
Acanthosis Nigricans in adults can be a sign of what? |
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Definition
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Term
Fibroepithelial polyp has a rare association with Birt-Hogg-Dube syndrome, which features what? |
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Definition
follicular lesions, kidney tumors, and pulmonary cysts |
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Term
What is an epithelial cyst typically filled with? |
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Definition
keratin and lipid-containing debris from sebaceous secretions |
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Term
What is unique about the pilar/trichilemmal cysts interms of their structure? |
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Definition
They lack a granular cell layer |
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Term
Where are pilar/trichilemmal cysts typically find? |
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Definition
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Term
Trichilemmomas are typically featured in what syndrome? |
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Definition
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Term
In sebaceous adenoma, what two cell populations are increased? What syndrome is sebaceous adenoma commonly linked to? |
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Definition
both sebocytesa and increased peripheral basaloid cells are prominent/ commonly linked to Muir-Torre sydrome, which also features HNPCC |
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Term
Squamous cell carcinoma commonly manifests as what? |
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Definition
sharply defined, red scaling plaques |
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Term
In the pathogenesis of SCC, what mutation is common? |
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Definition
p53, leading to loss G1 growth arrests and DNA repair |
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Term
In nevoid BCC syndrome, what happens as a result of the PTCH gene defect |
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Definition
causes uninhibited activation of SMO leading to BCC |
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Term
What distinguishes a benign fibrous histiocytoma from a malignant one? |
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Definition
Benign ones are factor XIIIa positive and CD34 negative |
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Term
In whom and on where does benign fibrous histiocytoma (dermatofibroma) occur? |
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Definition
on the legs of young-to-middle aged women |
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Term
Upon lateral compression, what happens to the dermatofibroma? |
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Definition
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Term
What are some histologic features of dermatofibroma? |
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Definition
overlying epidermal hyperplasia and entrapped collagen bundles |
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Term
What is the molecular hallmark of dermatofibrosarcoma protuberans? |
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Definition
balanced translocation between COL1A1 and PDGF Beta--> leading to overexpression of PDGF Beta (treat with TK inhibitor) |
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Term
Name two histologic features of dermatofibrosarcoma protuberans? |
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Definition
(1) invasion leading to trapping of fat and honeycombed appearance
(2) fibroblasts have radial arrangement- storiform pattern (like a pinwheel) |
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Term
In Sturge Weber syndrome, whom do we typically target for suspicion? |
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Definition
child with a large facial port-wine stain, seizures, and mental deficiency (stain a form of vascular ectasia) |
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Term
Mycosis fungoides may be confused with psoriasis for what reason? |
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Definition
it presents as raised, scaling plaques just like psoriasis |
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Term
In mycosis fungoides, what clusters are located in the epidermis? |
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Definition
T-helper cell aggregate bands called Pautrier microabscesses |
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Term
In sezary syndrome, there are the features of Mycosis Fungoides plus what else? |
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Definition
blood involvement leading to diffuse erythema and scaling |
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Term
A localized form of mastocytosis is known as what? how does it present? |
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Definition
urticaria pigmentosa/ lesions are round to oval, red-brown, nonscaling papules and small plaques |
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Term
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Definition
related to mastocytosis; area of dermal edema and erythea (wheal) in response to rubbing |
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Term
What's the pathogenesis of Mastocytosis and how do we use that information to treat the disease? |
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Definition
there are acquired activating point mutations in the c-KIT receptor tyrosine kinase leading to constitutive activation so we try to block that |
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Term
In acute inflammatory dermatoses what cell population is not present, surprisingly? |
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Definition
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Term
In urticaria, what's happening to the dermis? |
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Definition
there is edema formation with scattered eosinophils/neutrophils and dilated vascular spaces |
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Term
In acute eczematous dermatitis, what do the lesions look like? |
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Definition
red, papulovesicular, oozing, crusted lesions |
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Term
Spongiosis with intraepithelial vesicle formation occurs in what disease? |
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Definition
Allergic contact dermatitis |
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Term
In erythema multiforme, what is special about the location of the lesions? |
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Definition
they frequently are widely distributed, and there is symmetric involvement of the extremities |
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Term
What is the apperance of the lesion in erythema multiforme? |
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Definition
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Term
Accumulation of lymphocytes along the DEJ, intimate association with degenerating and necrotic keratinocytes is associated with with what disease? |
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Definition
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Term
What are two variants of Erythema multiforme that can be damaging? |
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Definition
Stevens Johnson Syndrome and toxic epidermal necrolysis |
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Term
In psoriasis, what happens to the stratum granulosum? |
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Definition
it becomes thinned/absent, with increased cell turnover and an extensive overlying parakeratotic scale |
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Term
Accumulations of neutrophils in the stratum corneum are known as what in Psoriasis? |
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Definition
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Term
With what genotype does psoriasis have a strong association? |
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Definition
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Term
What organism may have a possible role in seborrheic dermatitis? |
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Definition
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Term
What is known about the histologic features of seborrheic dermatitis? |
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Definition
they share features with both spongiotic dermatitis and psoriasis |
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Term
The Wickham striae in Lichen planus that highlight the papules are due to what? |
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Definition
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Term
In the dermoepidermal junction, what do we see in Lichen planus? |
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Definition
we see band-like infiltrates of lymphocytes at the DEJ, pointed rete ridges due to chronic basal cell layer injury by lymphocytes, and colloid/civatte bodies (anucleate, necrotic basal cells) incorporated into the inflamed papillary dermis |
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Term
What does IF of lupus reveal? |
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Definition
granular band of IgG and C3 at the DEJ |
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Term
What is icthyosis due to and what does it mean when it is acquired in adulthood? |
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Definition
It is due to defective desquamation and can signify lymphocytic or visceral malignancies when acquired in adults |
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Term
What happens to the stratum corneum in Ichthyosis? |
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Definition
it loses its basket weaving pattner- just parallel strips |
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Term
What's an example of a suprabasal blister? |
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Definition
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Term
What is the appearance of the vesicles in Pemphigus? |
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Definition
They are flaccid and accompanied by bullae and crust |
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Term
What sort of inflammatory cells do we predominantly see in pemphigus vulgaris? |
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Definition
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Term
In pemphigus folliaceous, what is rarely affected? |
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Definition
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Term
In Pemphigus IF, what is the pattern of the Ig deposition? |
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Definition
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Term
In bullous pemphigoids, what is the condition of the bullae? |
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Definition
they are tense and do not rupture easily |
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Term
In bullous pemphigoids, what are the blisters filled with? |
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Definition
mostly eosinophils and some lymphocytes assocatied with basal cell layer destruction and the creation of the subepidermal cleft |
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Term
In bullous pemphigoid, what is present along the DEJ? |
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Definition
there is basal vacuolization |
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Term
What causes DEJ separation in bullous pemphigoids? |
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Definition
When there is antibody binding to BPAG2 in the lamina lucida of the basement membrane, that activates complements and there is recruitment of eosinophil peroxidase and neutrophilic proteases. |
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Term
In dermatitis herpetiformis, what do the IgA autoantibodies bind to? |
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Definition
fibrils that anchor hemidesmosomes to dermis |
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Term
In Dermatitis Herpetiformis, where are the IgA deposits typically located? |
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Definition
at the tips of the Dermal Papillae |
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Term
What is the basis for epidermolysis bullosa? |
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Definition
there are inherited defects in structural proteins that stabilize desmosomes or hemidesmosomes |
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Term
In the simplex form of EB, what is the genetic basis? |
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Definition
there are mutations in the genes encoding keratins |
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Term
In the dystrophica type of EB, where are the genetic defects? |
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Definition
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Term
How is recessive EB different from AD EB? |
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Definition
it is more severe, there are scars and deformities of the teeth |
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Term
In porphyria, what are the primary alterations detected by light microscopy? |
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Definition
there is subepidermal blistering and marked thickening of the walls of superficial blood vessels |
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Term
In rosacea, there are high levels of what? |
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Definition
cathelicidn (endogenous antimicrobial peptide in high levels) |
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Term
In panniclitis, there is inflammatory reaction that affects what two sources? |
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Definition
connective tissue septa separating fat lobules or the fat lobules themselves |
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Term
In which type of panniculitis is there vasculitis? |
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Definition
erythema induratum--> granulomatous inflammation and zones of caseous necrosis involving the fat lobbule/ necrotizing vasculitis |
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Term
Name two histologic features of verruca vulgaris? |
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Definition
hypergranulosis with keratohyaline granules and koilocytic changes |
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Term
What is the unique feature of molluscum contagiosum papules? |
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Definition
They have central umbilication |
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Term
What is the general apperance of the lesion in a superficial fungal infection? |
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Definition
annular lesion with central clearing and fine peripheral scale |
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