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Skin Pathology
Exam 9 Material
93
Medical
Graduate
03/02/2011

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Term
What is unique about the melanocytes' cytostructure?
Definition
They have perfectly round nuclei
Term
In addition to manufacturing hair shafts, what else are hair follicles capable of doing?
Definition
harbor epithelial stem cells capable of regenerating superficial epithelial skin structures
Term
Where are the Merkel cells and Meissner corpuscles located, respectively?
Definition
w/in the epithelial basal cell layer/ in the papillary dermis
Term
What is lichenification and why does it occur?
Definition
It is the diffuse thickening of the epidermis with resultingg accentuation of skin lines. It occurs from intense, chronic rubbing.
Term
In which disease do we see marked acanthosis?
Definition
lichen simplex chronicus (often due to chronic irritation)
Term
What is the difference between pemphigus vulgaris and bullous pemphigoid?
Definition
The former deals with autoantibodies to intercellular keratinocyte connections. The latter deals with autoantibodies to dermoepidermal junction antigens.
Term
Dyskeratosis, the abnormal premature keratinization below the stratum granulum, typically occurs as a result of what?
Definition
drug reactions
Term
What is exocytosis?
Definition
Influx of inflammatory cells into the epidermis (spongiosis- intercellular edema of the epidermis)
Term
What is hyperkeratosis, and what disease features such change?
Definition
hyperplasia of the stratum corneum/ find this in ichthyosis
Term
What causes a wart?
Definition
hyperplasia and enlargement of contiguous dermal papillae
Term
What's the genetic basis of oculocutaneous albinism, and what are associating presentations?
Definition
AR, skin malignancies risk increased
Term
In addition to non-nested (linear) melanocytic hyperplasia, what else is commonly found in a lentigo?
Definition
elongated rete ridges
Term
What are the genetic variations that are associated with nevi being neoplasms?
Definition
mutations in BRAF or NRAS follwed by defect in p16/INk4a which normally arrests cyclin-dependent kinases
Term
What the basis behind the strong dark coloration of a blue nevus?
Definition
The melanocytes are deep down in the dermis, so that light gets poorly refracted
Term
Clinically, what is a spitz nevus often confused with?
Definition
hemangioma
Term
What are the cytologic features of Spitz nevus?
Definition
large, plump cells with pink-blue cytoplasm, spindle-shaped cells, pagetoid nefts and clefts around the nests
Term
In a halo nevus, why is there lightening of the skin around the nevus?
Definition
Because the infiltrating lymphocytes respond not only against nevus cells but also against surrounding normal melanocytes
Term
In dysplastic nevi, what is characteristic of the nevus cell nests?
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
Term
For a dysplastic nevus with cytologic atypia, what is occurring in the dermis?
Definition
There is linear/lamellar fibrosis (bridging fibrosis)
Term
In autosomal dominant dysplastic nevus syndrome, what are the two common mutations found?
Definition
mutated genes in CDKN2A and CDK4
Term
What does radial growth refer to?
Definition
horizontal spread of melanoma within the epidermis and superficial dermis
Term
In Lentigo Maligna, how does it usually present?
Definition
as an indolent lesion on the fae of older men that may remain in the radial growth phase for several decades
Term
How do you measure the Breslow Depth of Invasion?
Definition
from the granular cell layer to the base of the tumor
Term
For gauging the prognosis of a melanoma, at what depth above and below do we measure?
Definition
1.7 mm
Term
What are the 5 levels of invasion according to Clark?
Definition
Intraepidermal, Papillary, Papillary-Reticular, Reticular, and Subcutaneous
Term
Seborrheic Keratoses in people of color may manifest as color, multiple small lesions on the face?
Definition
Dermatosis papulosa nigra
Term
What is an association with Seborrheic Keratosis that does not involve the skin?
Definition
paraneoplastic syndrome- Leser Trelat
Term
Describe the appearance of the seborrheic keratosis?
Definition
well-demarcated coinlike pigmented lesion containing dark keratin-filled surface plugs
Term
What type of cysts are associated with seborrheic keratosis?
Definition
invagination and horn cysts
Term
Acanthosis Nigricans in adults can be a sign of what?
Definition
underlying GI cancers
Term
Fibroepithelial polyp has a rare association with Birt-Hogg-Dube syndrome, which features what?
Definition
follicular lesions, kidney tumors, and pulmonary cysts
Term
What is an epithelial cyst typically filled with?
Definition
keratin and lipid-containing debris from sebaceous secretions
Term
What is unique about the pilar/trichilemmal cysts interms of their structure?
Definition
They lack a granular cell layer
Term
Where are pilar/trichilemmal cysts typically find?
Definition
on the scalp
Term
Trichilemmomas are typically featured in what syndrome?
Definition
Cowden syndrome
Term
In sebaceous adenoma, what two cell populations are increased? What syndrome is sebaceous adenoma commonly linked to?
Definition
both sebocytesa and increased peripheral basaloid cells are prominent/ commonly linked to Muir-Torre sydrome, which also features HNPCC
Term
Squamous cell carcinoma commonly manifests as what?
Definition
sharply defined, red scaling plaques
Term
In the pathogenesis of SCC, what mutation is common?
Definition
p53, leading to loss G1 growth arrests and DNA repair
Term
In nevoid BCC syndrome, what happens as a result of the PTCH gene defect
Definition
causes uninhibited  activation of SMO leading to BCC
Term
What distinguishes a benign fibrous histiocytoma from a malignant one?
Definition
Benign ones are factor XIIIa positive and CD34 negative
Term
In whom and on where does benign fibrous histiocytoma (dermatofibroma) occur?
Definition
on the legs of young-to-middle aged women
Term
Upon lateral compression, what happens to the dermatofibroma?
Definition
inward dimpling
Term
What are some histologic features of dermatofibroma?
Definition
overlying epidermal hyperplasia and entrapped collagen bundles
Term
What is the molecular hallmark of dermatofibrosarcoma protuberans?
Definition
balanced translocation between COL1A1 and PDGF Beta--> leading to overexpression of PDGF Beta (treat with TK inhibitor)
Term
Name two histologic features of dermatofibrosarcoma protuberans?
Definition

(1) invasion leading to trapping of fat and honeycombed appearance

(2) fibroblasts have radial arrangement- storiform pattern (like a pinwheel)

Term
In Sturge Weber syndrome, whom do we typically target for suspicion?
Definition
child with a large facial port-wine stain, seizures, and mental deficiency (stain a form of vascular ectasia)
Term
Mycosis fungoides may be confused with psoriasis for what reason?
Definition
it presents as raised, scaling plaques just like psoriasis
Term
In mycosis fungoides, what clusters are located in the epidermis?
Definition
T-helper cell aggregate bands called Pautrier microabscesses
Term
In sezary syndrome, there are the features of Mycosis Fungoides plus what else?
Definition
blood involvement leading to diffuse erythema and scaling
Term
A localized form of mastocytosis is known as what? how does it present?
Definition
urticaria pigmentosa/ lesions are round to oval, red-brown, nonscaling papules and small plaques
Term
What is Darier sign?
Definition
related to mastocytosis; area of dermal edema and erythea (wheal) in response to rubbing
Term
What's the pathogenesis of Mastocytosis and how do we use that information to treat the disease?
Definition
there are acquired activating point mutations in the c-KIT receptor tyrosine kinase leading to constitutive activation so we try to block that
Term
In acute inflammatory dermatoses what cell population is not present, surprisingly?
Definition
neutrophils
Term
In urticaria, what's happening to the dermis?
Definition
there is edema formation with scattered eosinophils/neutrophils and dilated vascular spaces
Term
In acute eczematous dermatitis, what do the lesions look like?
Definition
red, papulovesicular, oozing, crusted lesions
Term
Spongiosis with intraepithelial vesicle formation occurs in what disease?
Definition
Allergic contact dermatitis
Term
In erythema multiforme, what is special about the location of the lesions?
Definition
they frequently are widely distributed, and there is symmetric involvement of the extremities
Term
What is the apperance of the lesion in erythema multiforme?
Definition
targetoid (target-like)
Term
Accumulation of lymphocytes along the DEJ, intimate association with degenerating and necrotic keratinocytes is associated with with what disease?
Definition
erythema multiforme
Term
What are two variants of Erythema multiforme that can be damaging?
Definition
Stevens Johnson Syndrome and toxic epidermal necrolysis
Term
In psoriasis, what happens to the stratum granulosum?
Definition
it becomes thinned/absent, with increased cell turnover and an extensive overlying parakeratotic scale
Term
Accumulations of neutrophils in the stratum corneum are known as what in Psoriasis?
Definition
Munro microbascesses
Term
With what genotype does psoriasis have a strong association?
Definition
HLA-C
Term
What organism may have a possible role in seborrheic dermatitis?
Definition
Malassezia furfur
Term
What is known about the histologic features of seborrheic dermatitis?
Definition
they share features with both spongiotic dermatitis and psoriasis
Term
The Wickham striae in Lichen planus that highlight the papules are due to what?
Definition
areas of hypergranulosis
Term
In the dermoepidermal junction, what do we see in Lichen planus?
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
Term
What does IF of lupus reveal?
Definition
granular band of IgG and C3 at the DEJ
Term
What is icthyosis due to and what does it mean when it is acquired in adulthood?
Definition
It is due to defective desquamation and can signify lymphocytic or visceral malignancies when acquired in adults
Term
What happens to the stratum corneum in Ichthyosis?
Definition
it loses its basket weaving pattner- just parallel strips
Term
What's an example of a suprabasal blister?
Definition
pemphigus vulgaris
Term
What is the appearance of the vesicles in Pemphigus?
Definition
They are flaccid and accompanied by bullae and crust
Term
What sort of inflammatory cells do we predominantly see in pemphigus vulgaris?
Definition
eosinophils
Term
In pemphigus folliaceous, what is rarely affected?
Definition
mucous membranes
Term
In Pemphigus IF, what is the pattern of the Ig deposition?
Definition
Net-like pattern
Term
In bullous pemphigoids, what is the condition of the bullae?
Definition
they are tense and do not rupture easily
Term
In bullous pemphigoids, what are the blisters filled with?
Definition
mostly eosinophils and some lymphocytes assocatied with basal cell layer destruction and the creation of the subepidermal cleft
Term
In bullous pemphigoid, what is present along the DEJ?
Definition
there is basal vacuolization
Term
What causes DEJ separation in bullous pemphigoids?
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.
Term
In dermatitis herpetiformis, what do the IgA autoantibodies bind to?
Definition
fibrils that anchor hemidesmosomes to dermis
Term
In Dermatitis Herpetiformis, where are the IgA deposits typically located?
Definition
at the tips of the Dermal Papillae
Term
What is the basis for epidermolysis bullosa?
Definition
there are inherited defects in structural proteins that stabilize desmosomes or hemidesmosomes
Term
In the simplex form of EB, what is the genetic basis?
Definition
there are mutations in the genes encoding keratins
Term
In the dystrophica type of EB, where are the genetic defects?
Definition
 In type VII collagen
Term
How is recessive EB different from AD EB?
Definition
it is more severe, there are scars and deformities of the teeth
Term
In porphyria, what are the primary alterations detected by light microscopy?
Definition
there is subepidermal blistering and marked thickening of the walls of superficial blood vessels
Term
In rosacea, there are high levels of what?
Definition
cathelicidn (endogenous antimicrobial peptide in high levels)
Term

In panniclitis, there is inflammatory reaction that affects what two sources?

Definition
connective tissue septa separating fat lobules or the fat lobules themselves
Term
In which type of panniculitis is there vasculitis?
Definition
erythema induratum--> granulomatous inflammation and zones of caseous necrosis involving the fat lobbule/ necrotizing vasculitis
Term
Name two histologic features of verruca vulgaris?
Definition
hypergranulosis with keratohyaline granules and koilocytic changes
Term
What is the unique feature of molluscum contagiosum papules?
Definition
They have central umbilication
Term
What is the general apperance of the lesion in a superficial fungal infection?
Definition
annular lesion with central clearing and fine peripheral scale
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