2. Skin Pathology Flashcards

1
Q

What cells (keratinocytes) are tough durable physical barrier, cells are attached to desmosomes, synthesize keratin, produce cytokines and defensins to regulate the cutaneous environment?

A

Squamous epithelial cells

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2
Q

Melanocytes produce melanin and protect from and screens UV light, langerhans cells are dendritic cells in the skin, process and present antigen to activate the adaptive immune system via migration to LN and present antigens to T cells, lymphocytes in the skin include CD4 CD8 and T reg cells, produce cytokines mediating the patterns and expression of inflammation and infectious disease. What nerves are responsible for physical sensations such as touch vibration pain itch cold and heat?

A

Afferent nerve fibers

*merkel cells are located in the epithelial basal layer without a known function

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3
Q

What are sweat glands which guard against variation of body temp and hair follicles which elaborate hair shafts with repositories for epithelial stem cells– they traverse epidermis and dermis?

A

Adnexal Skin components

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4
Q

Excoriation is linear, traumatic epidermal disruption often self induced. Lichenification is thick rough skin with prominent markings that can be due to repeated rubbing, oncholysis is separation of a nail from the underlying skin, a flat circumbscribed area are distinguised by coloration, what is it called when the falt lesion is less than or greater than 5mm?

A

less than 5mm is macule

greater than 5mm is patch

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5
Q

An elevated, dome shaped or flat topped lesions, if less than 5mm is a papule if greater than 5mm is a nodule, a plaque is an elevated flat topped lesions >5mm, may be caused by coalesced papules. Pustule is a discrete pus filled raised lesion, a scale is a dry horny plate like excrescence due to aberrant cornification, what is a pruritic elevated erythematous lesion with variable blanching, secondary to dermal edema?

A

Wheal**

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6
Q

Blisters have two types, fluid filled, raised lesions- vesicles are less than 5mm and bulla are greater than 5mm, acanthosis is diffuse epidermal hyperplasia, dyskeratosis is abnormal keratinization below the stratum granulosum, erosion is focal, incomplete discontinuous epidermal loss, what is infiltration of the epidermis by inflammatory cells?

A

Exocytosis

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7
Q

Hydropic swelling (ballooning) is intracellular keratinocyte edema often seen in viral infections, hypergranulosis is stratum granulosum hyperplasia usually due to rubbing, hyperkeratosis is stratum corneum thickening with abberant keratinization, lentiginous is linear non-nested melanocyte proliferation within the epidermal basal cell layer, what occurs when stratum corneum keratinization has retained nuclei- normally present on mucus membranes?

A

**parakeratosis

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8
Q

Papillomatosis is surface elevation due to dermal papillae hyperplasia, spongiosis is epidermal intercellular edema, ulceration is focal, complete epidermal loss reveals dermis or subdermis, vacuolization is vacuoles within or adjacent to keratinocytes, dysplatic nevus syndrome is related to a high risk of MM, what syndrome is associated with trichellomomas, and breast/endometrial and other cancers?

A

Cowden Syndrome

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9
Q

The 5 layers of the skin can be remembered by this mnemonic: Come, Lets Get Sun Burned - what are the layers?

A
Stratum Corneum
Stratum Lucidum (only on soles of hands and feet)
Stratum granulosum
Stratum Spinosum
Stratum Basale
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10
Q

What is the MC pigmented lesions of childhood in lightly pigmented individuals, 1-10mm, tan-red-brown macules, fade and recur depending of the amount of sun exposure, normal melanocyte density, hyperpigmentation due to focal melanin overproduction within basal keratinocytes (melanosomes)?

A

Freckles (ephelis)

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11
Q

There are major proteins formed within keratinocytes called keratins, which are intermediate filaments proteins making up the cytoskeleton of the cells, antibodies to various cytokeratins are used in pathology to identify keratinocytes. Maturation from basale to corneum is as follows: columnar to cuboidal to?

A

flattened

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12
Q

The epidermis is made of stratified squamous epithelium, the dermis is mainly connective tissue which contains blood vessels, nerves, and adnexa which is hair follicles, sebaceous gland and sweat glands (apocrine and eccrine), subcutaneous tissue mainly consists of ?

A

Fibrofatty tissues

NOTE: adnexa/appendages also include nails of toes and fingers

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13
Q

What is a common, benign, linear non-nested melanocytic hyperplasia in mucus membranes and skin, only in cell layer above BM- hyperpigmented basal cell layer, common in infancy and childhood, does not darken with sun exposure, hyperpigmented linear basal melanocyte hyperplasia, may see reet ridge elongation and thinning?

A

Lentigo (lentigines)

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14
Q

What is aka as a pigmented nevi or MOLE, common, congenital or acquired melanocyte neoplasms, most due to acquired activating mutations of RAS signaling*, more prominent during pregnancy indicated hormone sensitivity, rarely give rise to melanomas, p16/ink4a inhibits CDK4/6 = protective response?

A

Melanocytic Nevus

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15
Q

A junctional nevus occurs when there are nests of nevus cells at the dermoepidermal junctions, more common in kids and flat- most grow into the underlying dermis as nests or cords of cells to form compound nevi… What type of nevus is nests or cords of melanocytes extending into the underlying dermis (cells mature) becoming small and nonpigmented with fusiform contours and growing in fascicles, resembling neurological tissue = neurotization=b9? (note MM have no maturation)

A

Compound Nevus - both epidermal and dermal

intradermal nevus is completely dermis only

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16
Q

What nevus is a deep dermal subq growth around adnexa, neurovascular bundles and blood vessel walls, indentical to ordinary acquired nevi, present at birth, large variants = increase risk in MM?

A

Congenital Nevus

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17
Q

A blue nevus is a black blue nodule, non-nested dermal infiltration, often with assoc fibrosis, highly dendritic, heavily pigmented nevus cells, clinically may look like MM. What nevus has fascicular growth, fusiform cells, large plump cells with red pink blue cytoplasm, creates a red pink nodule, common in children, clinically confused with hemangioma?

A

Spindle and Epithelioid Cell nevus = SPITZ nevus

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18
Q

A halo nevus is identical to ordinary acquired nevi, lymphocytic infiltration surrounding nevus cells due to host immune repsonse against nevus cells and surrounding normal melanocytes, what nevus is described as coalescent intraepidermal nests, cytological atypia, potential marker or precursor of melanoma, may be direct precursors of MM when multiple in number are a marker of increased risk?

A

Dysplastic Nevus

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19
Q

Dysplastic Nevi are flat macules/slightly raised plaques with variable pigmentation and irregular borders in sun exposed and protected areas, most >5mm, may transform to MM may be direct precursor or increased risk, majority or stable and do not progress, due to loss of CDKN2A leading to CDK4 activation, NRAS and BRAF activation, TERT. Morphologically, what is characteristic in the dermis underlying atpical cells in a DN?

A

Linear or Lamellar fibrosis**

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20
Q

A DN can be a compound nevus with cytologic and architectural atypia, including irregular, angulated, nuclear contours with hyperchromasia, enlarged and fused (coalesced) nests of nevus cells, linear papillary dermal fibrosis is common due to melanin incontinence where melanin is lost and eaten by macrophages, what hyperplasia causes replacement of the basal cell layer along the dermoepidermal junction?

A

Lentiginous melanocyte hyperplasia

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21
Q

What syndrome is AD, develops multiple DN and MM are co inherited, develop multiple (hundreds), 50% have MM by age 60, due to loss of function of CDKN2A- different from typical melanocytic nevi- which encodes p16/ink4a (negative regulator of CDK4/6), most lesions dont progress- need more risk factors for cancer?

A

Dysplastic Nevus Syndrome

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22
Q

What is the most deadly of all skin cancers, cured if detected early and resected, strongly linked to UV radiation, most arise in skin, atypical melanocytes in nests and single cellular arrays at the dermal epidermal junction?

A

Malignant Melanoma

**worse and MC in men- cause men dont like drs duh

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23
Q

Cutaneous melanomas are due to UV damage causes DNA damage- UVB is the worst, risk includes severe sunburn in early life, lightly pigmented, fam hx mm, MC on back, arms, neck, legs, females back/legs, males upper back, morphology shows variations in color, on histo you see large cells with expanded, irregular what, containing peripherally clumped chromatin and prominent red nucleoli?

A

irregular Nuclei**

*stain with HMB-45

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24
Q

Radial growth of MM involves horizontal spread o MM in the epidermis and superficial dermis, unable to metastasize- 3 types include lentigo maligna, superficial spread is MC, and acral/mucosal lentiginous MM, what type of growth may occur after radial or horizontal growth, has met potential, tumor subclone with appearance of nodule, see dermal invasion of cells without maturation/neurotization, cells have met capabilities that increase as depth of invasion increases (breslow thickness)?

A
Vertical growth
probability of metastasis correlates with depth of invasion
•	<1mm: 95-100% 5y survival
•	1-2mm: 80-96% 5y survival
•	2.1-4mm: 60-75% 5y survival
•	>4mm: 37-50% 5y survival
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25
Q

What is a benign epithelial tumor that is common spontaneous, arise on trunk most commonly, middle aged or older individuals, due to activating mutations in FGFR3, see a rounnd, flat coin like waxy plaque, sharply demarcated, uniform, tan brown velvety or granular round plaques see hyperplasia of pigmented basaloid cells and hyperkeratosis, keratin filled plugs may be seen along with keratin filled horn cysts, if irritated and inflamed basaloid cells undergo whirling foci of squamous differentiation?

A

Seborrheic keratosis SK

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26
Q

What sign is characterized by a sudden appearance of multiple seborrheic keratoses and paraneoplastic syndrome (usually GI cancer), due to keratinocyte stimulation via TGFa produced by tumor cells?

A

Leser Trelat Sign

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27
Q

What is a benign epithelial tumor characterized by hyperpigmentaed skin with a velvety texture seen in flexural areas, sign of underlying conditions, due to increased growth factor receptor signaling in the skin, 80% benign assoc with obesity or DM, pineal or pituitary tumor, if malignant assoc with GI adenocarcinomas- paraneoplastic phenomenon?

A

Acanthosis Nigricans

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28
Q

What is a benign epithelial tumor is very common cutaneous lesion, found on neck trunk face, sporadic, see soft flesh colored tumors w fibrovascular stalk, covered in benign epidermis, may cause pain by torsion, clinically usually inconsequential, may be assoc w DM, obesity or intestinal polyposis, increase in # during pregnancy due to hormonal stimulation?

A

Fibroepithelial polyp = acrochordon = skin tag

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29
Q

What cyst is invagination and cystic expansion of epidermis or a hair follicle, filled with keratinaceous material, common lesions, if large + trauma can spill keratin into the dermis and may lead to an extensive and painful granulomatous inflammatory response?

A

Epithelial or Follicular Inclusion Cyst = Wen = Sebaceous cyst = circumscribed with capsule*

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30
Q

The following are what kind of benign tumors… eccrine poroma, cylindroma, syringoma, sebaceous adenoma, pilomatricoma + hundreds more?

A

Adnexal appendage tumors

Note: syringoma has eccrine differentiation, usually multiple, small, tan papules near the lower eyelids

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31
Q

Ataxia telangiestasia is AR 11q22.3 mutation of ATM gene causing neurologic and vascular lesion, Cowden is AD 10q23 PTEN mutation leading to b9 follicular appendage tumors = trichilemmomas and internal adenocarcinoma, what familial syndrome is AD 9q22 PTCh mutation causing multiple basal cell carcinomas, medulloblastomas and jaw cysts?

A

Nevoid basal Cell carcinoma sydrome

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32
Q

What syndrome is AD 9p21 mutation of CDK2/p16/ink4 or CDKN2/p14/ARF which inhibits RB promoting cell cycle arrest/melanoma/pancreatic carcinoma, binds MDM2 promoting p53 function leading to melanoma and pancreatic carcinoma?

A

Familial melanoma syndrome

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33
Q

What premalignant epidermal tumor is hyperkeratosis of sun damaged skin, increased incidence in lightly pigmented patients, assoc with cutaneous horns due to exuberant keratin production on the face arms and hands, and actinic cheilitis which is seen on the lips, see tan brown red flesh colored lesions, rough consistency, atypical basal cells due to keratosis, INTRACELLULAR BRIDGES ARE PRESENT - cant be seen in BCC?

A

Actinic (solar) Keratoses

34
Q

Actinic (solar) Keratoses are associated with elastosis which is thickened blue gray elastic fibers of the superficial dermis probably due to elastic fiber prodcution by sun damaged fibroblasts, see progressive dysplastic changes over time to SCC, what is seen which is thickened stratum corneum that retain their nuclei (unlike normal cells in this layer)?

A

Parakeratosis

35
Q

What carcinoma is the 2nd MC tumor of sun exposed skin of older individuals, males>females, invasive are usually small and resectable, <5% metastasize, biggest risk factor is lifetime sun exposure, comes from precursor AK, UV light causes DNA damage, associated with tp53 mutations, ATR and ATM loss increases incidence of repair via error prone mechanisms passed to daughter cells- commonly affects pyrimidine dimers ***?

A

Squamous cell carcinoma

36
Q

Squamous cell carcinoma in situ is well demarcated, red scaling plaques, invasive is nodular, vary from well differentiated with prominent keritinization to highly anaplastic with necrosis and ulceration, grossly is elevated, wart like and has irregular borders, what can be seen histologically if invasive?

A

extension past the basement membrane and inflammatory cells

37
Q

What is due to inherited mutations (AR) of the nucleotide excision repair of pyrimidine dimers, defective nucleotide excision repair leads to error prone repair, NER is required for accurate repair of pyrimidine dimers, rapid accumulation of mutations and carcinogenesis (SCC) younger patients with skin cancer is commonly seen?

A

Xeroderma Pigmentosum

38
Q

What is the MC invasive cancer in humans, MC malignancy worldwide, associated with activating mutations of the Hedgehog pathway signaling***, slow growing and rarely metastasize, due to sun exposed sites in lightly pigmented elderly adults, inc incidence with immunosuppression and dna repair disorders?

A

Basal Cell Carcinoma BCC

39
Q

Basal Cell Carcinoma BCC is due to uncontrolled hedgehog signaling- PTCH loss of function mutation which is a receptor for SHH, treatments targeting SHH pathway have excellent response rates, grossly see pearly papules with prominent telangiectatic vessels- what is commonly used to describe it, which is ulceration of advanced lesions that show extensive local invasion?

A

Rodent ulcers

pt commonly presents with a cut that isnt healing

morph- see islands of basophilic cells, hyperchromatic nuclei in lymphocytes and fibroblasts

40
Q

What is a tumor of the dermis is a heterogenous family of benign dermal neoplasms of uncertain lineage, factor XIIIa positive dermal dendritic cells, found on legs of young and middle aged adults, asymptomatic or tender may increase or dec over time, some pt had trauma in same area before, are firm brown tan papules tender with inward dimpling?

A

Dermatofibroma or Benign Fibrous Histiocytoma

41
Q

Dermatofibroma or Benign Fibrous Histiocytoma is the MC form, with spindle shaped fibroblasts in a well defined mid dermal non encapsulated mass, the are arranged in a circumscribed lenticular shape in the dermis but no capsule, overly epidermis shows pseudoepitheliomatous hyperplasia which is downward elongation of ?

A

hyperpigmented rete ridges (epidermal ridges)

42
Q

What is a malignant dermis tumor that is well differentiatied primary fibrosarcoma of the skin, slow growing, locally aggressive- rarely metastasizes due to translocation of COL1A1 and PDGFB, overexpression of PDGFB leads to tumor cell growth through an autocrine loop, tx with excision, morphology can see storiform pattern which are closely packed fibroblasts arranged radially (pinwheel appearance)?

A

Dermatofibrosarcoma Protuberans

43
Q
Mnemonic for painful skin lesions: GLENDAB
Glomus tumor
Leiomyoma (angio type)
Eccrine spiradenoma
Neurofibroma
Dermatofibroma
Angiolipoma
Blue rubber bleb nevus
A

yay

44
Q

What is a cutaneous T cell loma- lymphoma of skin homing CD4 T cells that presents in the skin,most remain localized to the skin for years but can evolve to systemic lymphoma (sezary syndrome), MC in patients older than 40 years, appear as scaly red brown patches on the trunk, raised scaling plaques may be confused with psoriasis, fungating nodules common?

A

Mycosis Fungoides

45
Q

Mycosis Fungoides is a tumor of cellular migrants to the skin, early looks like eczema like lesions which are raised indurated irregularly outlined erythematous plaques eventually multiple large red brown nodules indicate systemic disease prognosis is based on the percent of body surface involved and progression of patch to?

A

plaque to nodule

Sézary-Lutzner cells (hallmark): Malignant CD4+ T helper cell with a hyper-convoluted (cerebriform) nuclei
-form bandlike aggregates within the superficial dermis and invade the epidermis as single cells and small clusters (Pautrier microabscesses)

46
Q

What is a tumor of cellular migrants to the skin and is a spectrum of rare disorders characterized by increased number of mast cells in the skin, it is termed urticaria pigmentosa in children accounting for 50% of all cases, 10% have systemic disease with organ involvement typically in adults with a poor prognosis, systemic dz presents as pruritis flushing watery nasal discharge, bone pain (osteoporosis due to increase histamine in marrow, esp in premenopausal women = ***clue to dx)?

A

Mastocytosis

47
Q

Mastocytosis is typically single lesions, signs and sx related to release of histamine, heparin, alcohol etc when mast cells degranulate, path- acquired activating KIT receptor tyrosine mutations leading to mast cell growth and survival, see single pink tan brown nodule that may be pruritic or show blister formation, look for spindle and stellate cells with toluidine blue or giemsa stain to see granules. Darier sign is localized area of dermal edema and erythema (wheal) that occurs when lesional skin is rubbed, what is an area of dermal edema resembling a bee hive that occurs due to localized stroking of apparently normal skin with a pointed object?

A

Dermatographism

48
Q

What is a disorder of epidermal maturation with chronic excessive keratin buildup (hyperkeratosis) = fish like scales- defective desquamation leading to retention of abnormally formed scale, build up of stratum corneum with loss of nl basket weave pattern, minimal inflam, most present at or near time of birth, sometimes can be acquired and may be paraneoplastic, associated with lymphoid and visceral malignancies?

A

Ichthyosis (vulgaris)

49
Q

What inflam dermatoses last days to weeks, inflam infiltrates are lymphocytes, macrophages, see edema, variable degress of epidermal, vascular and subq injury, more commonly encountered and include urticaria, eczematous dermatitis, and erythema multiforme?

A

Acute Inflammatory Dermatoses

50
Q

What inflammatory dermatoses lasts months to years, assoc with changes in epidermal growth such as atrophy or hyperplasia or dermal fibrosis, skin may be roughened due to scale formation and shedding, not all scaling lesions are inflammatory- includes psoriasis, seborrheic dermatitis and lichen planus?

A

Chronic inflammatory dermatoses

51
Q

What is localized mast cell degranulation leading to dermal microvascular hyperpermeability- associated with wheals which is a firm edematous plaque resulting from infiltration of the dermis with fluid - they are transient and may last only a few hours (hives), it is the most common result of antigen induced release of vasoactive mediators from mast cells?

A

urticaria- type 1 hypersensitivity

angioedema is closely related and characterized by edema of the deeper dermis and the subcutaneous fat

52
Q

Urticaria (hives) can also be due to mast cell dependent IgE independent which results from substances that directly incite the degranulation of mast cells such as opiates abx, curare and contrast media. Urticaria is also due to mast cell independent IgE independent triggered by local factors that increase vascular permeability due to drugs or what, which is caused by an inherited def of C1 inhibitor that results in excessive activation of the early components of the complement system and production of vasoactive mediators?

A

Hereditary Angioneurotic edema (C1 esterase inhib)

histo for uticaria see superificial dermal edema, manifested by spaces between collagen bundle sand dilated lymphatic and blood filled vascular spaces- epithelium is normal

53
Q

What acute dermatitis is one of the most common skin disorders, cause may be inside due to circulating antigen from ingested foot or drug or outside due to external application of an antigen like poison ivy, tx with steroids nonspecifically block the inflam response and is palliative not curative, cure via removal of offending agent, all types characterized by red, papulovesicular, oozing, crusted lesions that develop acanthosis and hyperkeratosis that produce raised and scaling plaques?

A

Acute eczematous dermatitis

54
Q

Acute eczematous dermatitis is due to T cell mediated inflam reactions (type IV), langerhan cells present normal antigens that have been transformed into neoantigens, presentation allows CD4 to become memory and effector cells, re exposure occurs and release of cytokines by memory cells leads to acumm of inflam within 24 hours, lesions are prone to bacterial infection which causes yellow crust, acute eczema is characterized by what, which is edema seepins into intracellular spaces of epidermis splaying keratinocytes in stratum spinosum?

A

epidermal spongiosis (edema*- note urticaria was dermal not epi),

also see acantholysis

55
Q

What is an uncommon self limited type IV hypersensitivity reaction leading to a variety of lesions, assoc with mycoplasma pneumoniae infection.. causes include: infections such as herpes, histo, coccidioidomycosis, typhoid, leprosy, exposure to certain drugs, cancer, and collagen vascular diseases (any AI dz like lupus PAN, etc)?

A

Erythema Multiforme

*targetoid lesions- red ,maculopapular lesions with central pallor due to necrosis of keratinocytes with perivenular inflam = interface dermatitis

56
Q

Erythema Multiforme causes keratinocyte injury mediated by skin homing CD8 cytotoxic T cells- most prominent in central portion of lesions and CD4 cells / langerhans most prominent in peripheral area of lesion, presents as macules, papules vesicles, bullae, limited cases often show suymmetric involvement of extremities. What is a severe febrile form seen in children in response to drugs?

A

Steven Johnson Syndrome

57
Q

What is a chronic inflam dermatosis that appears to have an autoimmune basis, seen in patients of all ages, 15% have arthritis that may produce severe deformities, may affect a single joint or may be symmetrical, also associated with myopathy, enteropathy and AIDS?

A

Psoriasis

58
Q

2/3 of pt with psoriasis have HLA C gene, sensitized CD4, Th17 and CD8 cells accumlate in the epidermis and driver keratinocyte proliferation via elaboration of cytokine soup dominated by Th1/17, koebner phenomenon is when psoriatic lesions appear in susceptible patients due to local trauma, lesions affect skin of elbows knees scalp, variations include annular linear gyrate or serpiginous, one cause of total body erythema and scaling known as erythroderma lesions are what colored covered by loosely adhereing?

A

pink to salmon colored plaques covered by loosely adhering silver white scales

59
Q

Morphologically, see yellow brown nail changes with pitting dimpling and separation of nail plate from bed (onycholysis), see acanthosis (epidermal thickening), regular downward elongation of rete ridges looks like test tube rack, stratum granulosum is thinned or absent with extensive overlying parakeratotic scaling, what sign is when dilated vessels of underlying dermal papillae leading to multiple minute bleeding points when the overlying scaling is removed?

A

Auspitz Sign

psoriasis treated with anti TNF or anti IL17

60
Q

What is more common than psoriasis, inflam of the epidermis- not sebaceous glands but involves areaswith high densities of these glands including scalp forehead, auditory canal, nasolabial folds, path is unknown but increased sebum production in response to androgens, see macules and papulse on an erythematous yellow greasy base with scaling and crusting, severe in pt with HIV low CD4 counts and parkinsons disease d/t DA def?

A

Seborrehic Dermatitis

dandruff is common scalp lesion

**spongiotic dermatitis and acanthosis with parakeratotic mounds at the ostia of hair follicles = follicular lipping, and mixed inflam cell populations

61
Q

What is a chronic inflam dermatitis associatd with 6 P’s including, pruritic, purple polygonal planar papules and plaques (coalescence of papules), is typically self limited and resolves 1-2 yrs, resolution often leaves a residuum of post inflam hyperpigmentation, oral lesions persist for years, in which SCC may occur, koebner may occur as well, path is unknown?

A

lichen planus

62
Q

lichen planus has wickham striae which are white dots or lines that highlight the papules, lesions are mutliple and symmetric on wrists elbows and penis, oral lesions are white and lace like*, dermal epidermal junction takes on an angulated zigzag contour (**saw toothing), chornically see acanthosis, hyperkeratosis and thickening of granular cell layer, what bodies are necrotic basal cells sloughed off and incorporated into the inflamed papillary dermis?

A

Colloid/Civatte Bodies

63
Q

What diseases produce dramatic lesions and in some instances are fatal if left untreated, blisters in various disorders occur at different levels within the skin, histologic assessment is *essential for accurate diagnosis, usually caused by acquired or inherited defects in proteins that make up desmosomes and hemidesmosomes- includes pemphigus, bullous pemphigoid, and dermatitis herpetiformis?

A

Blistering (Bullous) Diseases

64
Q

What is an inflammatory blistering disorder due to autoantibodies that lead to the dissolution of intercellular attachments within the epidermis and mucosal epi, 4-60s no gender, usually benign, tend to rupture, many types, with vulgaris being MC (suprabasilar), foliaceus (subcorneal), AI disorder due to ***IgG autoabs against desmogleins 1/3 which disrupt intercellular adjesions and result in formation of blisters?

A

Pemphigus

65
Q

Pemphigus you see acantholysis, vulgaris is the MC type in which IgG deposits are seen at all levels of the epithelium, but is a suprabasilar acantholytic blister*, pemphigus vegetans is rare large moist verrucous plaquewith overlying epidermal hyperplasia, what type of pemphigus is benign and mild, rare affects mucous membranes, IgG deposits are superficial with blisters found at level of stratum granulosum?

A

Pemphigus Foliaceus

66
Q

NOTE: subepidermal- NON-acantholyic blisters = bullous pemphigoid
epidermal - acantholytic blisters = pemphigus

A

woof

67
Q

What is an AI blistering disease of skin and mucosa affecting inner thighs, flexor surfaces of forearms, axillae groin and lower abdomen, oral lesions appear after skin lesions, typically in ELDERLY and tend NOT to rupture, abs against BPAGs (blistering) that adhere basal keratinocytes to the BM = hemidesmosomes, linear depostion at dermoepidermal jxn, assoc with neutrophils, eosinophils and inflam?

A

Bullous Pemphigoid

Subepidermal, non-acantholytic blisters with linear dermoepidermal junction staining for Ig and complement
-Variable, superficial, perivascular dermal inflammatory cell infiltrate + degranulated eosinophils seen directly beneath basal epithelial cells

68
Q

What type of pemphigus is Associated with various malignancies, most commonly non-Hodgkin Lymphoma, (and GI cancers?) Caused by autoantibodies that recognize desmogleins or other proteins of intercellular adhesion?

A

paraneoplastic pemphigus

69
Q

What is urticaria grouped in vesicles with male predominance in 30-40s, some cases associated with intestinal celiac disease and respond to gluten free diets, pt has developed IgA abs to dietary gluten (gliadin), abs cross react with reticulin which anchors fibrils that tether the epidermal basement membrane to superficial dermis leading to a true subepidermal blister?

A

Dermatitis Herpetiformis

70
Q

Dermatitis Herpetiformis presents as plaques and grouped vesicles which are bilat and symmetrical on extensor surfaces, knees elbows upper back and butt, see neutrophils and fibrin accum in tips of dermal papillae, leading to small micro absecesses and on immunofluoresence shows discontinuous what ?

A

granular deposits of IgA at the tips of dermal papillae

71
Q

What is a non-inflam disorder that blister due to pressure rubbing or trauma caused by inherited defects of structural proteins that lend mechanical stability to the skin, blisters occur at or after birth*, EM is needed to determine the specific types (there are 4)?

A

Epidermolysis Bullosa

72
Q

What refers to a group of uncommon inborn or acquired distrubances of porphyrin metabolism, they are pigments that are normall present in hemoglobin, myoglobin, and cytochromes, its classification is based on both clinical and biochemical features- cutaneous manifestations consist of urticaria, and vesicles associated with scarring -exacerbated by exposure to sunlight?

A

Porphyria

73
Q

Non inflammatory acne is open comedones and closed comedones which are follicular papules, with black keratin plugs or no visible central plug respectively, acne vulgaris mainly due to keratin plug blocking outflow of sebum to skin surface + hypertrophy of sebaceous glands during puberty, lipase synthesizing bacteria…

A

meow

74
Q

What is Nonspecific perifollicular lymphocytes infiltrate with dermal edema and telangiectasia, Common disease of middle age and older, Females predominance, 4 stages include flushing, persistent erythema, pustules and papules due to neutrophil colonize follicles causing granulomatous response, and 4th stage is rhinophyma which is permanent thickening of nasal skin via confluent erythematous papules associated with hypertrophy of sebaceous glands and follicular plugging by keratotic debris?

A

Rosacea

75
Q

Rosacea has high cutaneous levels of cathelicidin mediates cutaneous innate immunity, cathelicidin peptides present are qualitatively distinct from those seen in patients without rosacea as a result of alternative processing by proteases such as kallikrein 5, activation of what causes upregulation of kallikrein 5 in keratinocytes = disease?

A

TLR2

76
Q

What is inflammation of subcutaneous adipose tissue that affects the lobules of fat or connective tissue that separates fat into lobules often involving the lower legs, with two distinctive forms including erythema nodosum (MC subacute presentation) and erythema induratum - uncommon?

A

Panniculitis

77
Q

What type of panniculitis is poorlt defined, tender, erythematous plaques and nodules that are readily palpable, not easily seen, over weeks the lesions flatten and and become bruise like and leave no scars, may also have fever malaise due to delayed hypersensitivity type IV reaction to microbial or drug related antigen- 60% dont know the cause, associated with **SARCOIDOSIS*= non-necrotizing granulomas IBD?

A

Erythema Nodosum

*dx via bx of deep wedge of tissue to generously sample the subcutis - required

78
Q

Verrucae are warts due to HPV, self limited that affect children and adolescents, HPV 6/11 = anogenital, 5/8 SCC, 16= SCC of genitalia, E6 kill p53 and E7 kill RB, see koilocytosis which is cytoplasmic vacuolization involving the more superficial epidermal layers. What is the MC type of wart occurs anywhere but MC on hands or periungal, are gray white tan and flat to convex papules with rough pebble like surface?

A

Verruva vulgaris

  • plana are flat warts
  • plantaris/palmaris on hands/feet
  • condyloma accuminata- genital lesions
79
Q

What is a common self limiting viral skin disease due to poxvirus*, characterized by brick shaped with dumbbell shaped DNA, spread via contact, likes trunk and anogenital, children and young adults, lesions are firm pruritic umbilicated pink papules with diagnostic molluscum bodies on giemsa- inclusions in cells of stratum granulosum and stratum corneum?

A

Molluscum Contagiosum

80
Q

What is common sup. bacterial infxn of face and hands, due to Staph Areus, blister forms due to bacterial toxin that cleaves desmoglein 1 so cell cell adhesion in uppermost epidermal layer is broke (like p. foliaceus), no dermis involved, see many small pustules with erosions and honey colored crust, accumulation of neutrophils beneath the stratum corneum***?

A

Impetigo (Innate immune response → epidermal injury –> local serous exudate and formation of a scale crust (scab))

81
Q

What infections are confined to the stratum corneum caused by dermatophytes that grow in the soil and on animals, fungi stain bright pink red with PAS and are found in the anucleate cornified layer of lesional skin hair or nails?

A

Superficial fungal infections
Tinea capitis/barebae (beard), corporis (body), cruris (inguinal obese men warm weather), pedis (atheletes foots most inflam is related to bact superinfection)

82
Q

What type if tinea occurs on the upper trunk and is due to malassezia furfur yeast, distinct appearance such as groups of macules of variable size with fine peripheral scale- lesions of hyper and hypopigmentation?

A

Tina versicolor