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AAD ETAS > systemic > Flashcards

systemic Flashcards

1
Q

xlr acral paresthesia renal failure

A

Fabry

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2
Q

alpha galactosidase a defect

A

Fabry

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3
Q

pityriasis rotunda is an indication of ?

A

paraneoplastic

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4
Q

tripe palms

A

Hcc scc

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5
Q

angiolipomas

A

tuberous sclerosis

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6
Q

stk11 serine threonine kinase

A

peutz jeghers

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7
Q

NHL CLL Thymoma castlemans tumor

A

paraneoplastic pemphigus

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8
Q

thyroid malignancy

A

papillary adneoca

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9
Q

rothmund thompson bloom werner xp

A

increased systemic maligancy

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10
Q

neurologic

A

riga fede

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11
Q

KI inhibit

A

thyroid hormone synthesis

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12
Q

paraneoplastic DIF

A

granular C3

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13
Q

c-anca

A

wegeners

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14
Q

pyostomatitis vegetans

A

crohns disease

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15
Q

eruptive xanthoma type of hypertriglyceridemia plane palmar xanthoma

A

1,4,5

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16
Q

turcot syndrome is associated with?

A

colon ca

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17
Q

NSF? paraproteinemia

A

no

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18
Q

HSP DIF

A

c3 fibrin

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19
Q

porphryin wavelength

A

400-410 nm

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20
Q

birt hogg dube-

A

trichodiscoma fibrofolliculoma fibroepithelial polyp

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21
Q

carcinoid syndrome- hi urine

A

5hydroxyindolacetic acid-somatastatin

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22
Q

HCV alcohol estrogens polyhalogenated HC

A

PCT

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23
Q

HCV pathogenesis PCT

A

uro decarboxylase deficiency iron stores decompartmentalisation

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24
Q

plexiform neuroma NF1 JXG,

A

JCML

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25
Q

EPP absorption in soret band

A

protoporphyrin 9-

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26
Q

fecal coproporhyrinogen

A

VP

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27
Q

porphyria no skin

A

AIP

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28
Q

angiofibroma collagenoma

A

MEN1

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29
Q

RET protooncogene

A

MEN 1-2 AD,

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30
Q

multiple mucosal neuromas

A

MEN 2b

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31
Q

lichen macular amyloidosis

A

MEN2a sipple syndrome

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32
Q

no increased SCC but photosensitivity

A

IBID

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33
Q

HLA DQ2/DR3/B8

A

DH

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34
Q

all gluten sensitive enteropathy

A

DH

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35
Q

DH thyroid association

A

Graves

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36
Q

urine 5HIAA

A

carcinoid syndrome

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37
Q

scleroderemoid changes are seen in which syndrome

A

carcinoid syndrome

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38
Q

cyrproheptadine

A

carcinoid syndrome

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39
Q

honeycombed PPK star shaped keratosis pseudoainhaim

A

vohwinkel

•

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40
Q

shoulder pad sign

A

systemic amyloidosis

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41
Q

DMS - men

A

(lymphoma)

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42
Q

Sclerodermatous thickenings

A

PCT

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43
Q

tuberin harmatin

A

TS bournavilles dz

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44
Q

an early sign of Gardner’s syndrome

A 
Congenital hypertrophy of
retinal epithelium (CHRPE)
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45
Q

AR pili torti deafness

A

Bjornstadt

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46
Q

NLD % DM pt

A

<5%

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47
Q

childbearing TS bournavilles dz

A

pulmonary lymphagioleiomyoma

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48
Q

hyperkeratotic spicules

A

MM

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49
Q

blepharitis, symblepharon, ectropion NL

A

Cockayne Syndrome

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50
Q

Cockayne Syndrome

A

AR NER

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51
Q

lentiginese adenomatous GIT polyps weight loss onycholysis

A

• cronkhite canada

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52
Q

protein losing enteropathy

A

cronkhite canada

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53
Q

rate limiting protoporphyrinogen

A

ALA synthetase

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54
Q

only oxidized pophyrin

A

dALA -

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55
Q

Deafness,retinal atrophy, basalganglia calcifications

A

Cockayne Syndrome

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56
Q

terrys nails

A

cirrhosis,

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57
Q

necrobiotic xanthogranuloma commmon site

A

periorbital

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58
Q

necrobiotic xanthogranuloma aw

A

monoclonal gammopathy

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59
Q

trichofollicloma trichodsicoma RCC Ptx

A

BHD syndrome-

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60
Q

hep C type virus

A

SsRNa

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61
Q

coloboma part of which syndrome

A

BCC nevus S

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62
Q

Erythema Gyratum Repenscommonly linked with

A

gastric CA

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63
Q

risk of lhermitt duclos is increased in which syndrome

A

cowdens syndrome

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64
Q

SCC oropharyngeal

A

Bazex

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65
Q

APS ab+LL

A

NFD

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66
Q

ESRF hyperparathyroidism raised ca po4

A

calciphylaxis

67
Q

wolf chiakoff effect

A

KI

68
Q

Montgomery S -

A

xanthoma disseminatum

69
Q

premature atherosclerosis

A

werner-

70
Q

earliest NF-

A

CALM

71
Q

NF prepuberty

A

, LIsch nodules

72
Q

postpuberty

A

neurofibroma

73
Q

HI-stool isocoprophyrin

A

HEP PCT

74
Q

A falsely high ankle brachial pressure
index occurs in patients with diabetes mellitus caused by
d The toeebrachial pressure index should be
measured in patients with diabetes mellitus
whenever possible, and the toe pressure
should be [55 mm Hg for adequate peripheral
circulation to heal

A

glycosylation or
calcification leading to noncompressible
vessels)

75
Q

ankle brachial pressure index value that indicate severe arterial disease and represents a significant
risk for delayed healing or a nonhealable
wound

A

<0.4

76
Q

toe pressure value that indicate severe arterial disease and represents a significant
risk for delayed healing or a nonhealable
wound

A

\30 mm Hg,

77
Q

pattern of flow
by handheld Doppler ultrasound that indicate severe arterial disease and represents a significant
risk for delayed healing or a nonhealable
wound

A

monophasic flow

by handheld Doppler ultrasound

78
Q

criterion standard for

evaluation of limb perfusion in persons with diabetes

A

Segmental continuous wave Doppler examination

and toe pressure

79
Q

Patients with FAP and Gardner syndrome arepredisposed to what malignancy

A

duodenal, thyroid, brain (me-dulloblastomas), adrenal, and liver cancers

80
Q

most common malignancy in FAP.

A

Colon, then Duodenal cancer is thesecond, thyroid, adrenal, hepatoblastoma, medulloblastoma

81
Q

For gardner/FAP,

Malignancy screening colon CA should begin at

A
  1. 10 to 15 years ofage for classic FAP, and 18 years of age for attenu-ated FAP until the recommendation for surgery ismade.
    Upper GI endoscopy is recommendedevery 1 to 3 years beginning at 25 to 30 years
  2. annual thyroid examination increased riskof thyroid cancer.
82
Q

PeutzeJeghers syndrome (PJS) is caused by agermline mutation in the STK11 gene present on chromosome

A

19p13.3

83
Q

The most common malignancies in patientswith ? are small intestinal, colorectal, gas-tric, and pancreatic adenocarcinoma
breast, uterus, cervix, andtestes.
Breast cancer risk is of particular concern,afflicting 32% to 54% of patients.48,49 Malignantmelanoma is rarely reported.

A

PJS

84
Q

The diagnosis of PJS requires the presence

A

ofhistopathologically confirmed hamartomatous polyps
2 out of 3 additional criteria, including character-istic mucocutaneous pigmentation, a specific type ofGI polyp, and a family history of PJS.

85
Q

phosphatase and tensin homolog hamartom-atous tumor syndrome

A

Cowden syndrome

Bannayan-Riley-Ruvalcaba syndrome

86
Q

Breast, colon, and thyroid cancers are mostcommon malignancies associated with

A

Cow-den syndrome

87
Q

.BRRS the most specific cutane-ous finding.

A

Hyperpigmented macules involving the glanspenis or vulva

88
Q

hamartomatous gastrointestinalpolyps, macrocephaly,
hemangiomas, developmental delay
Which syndrome?

A

BRRS

89
Q

Ocular abnormalities BRRS

A

involving the retina (Schwalbelines) and cornea occur in 35%of patients.

90
Q

Neurofibromatosis has been linked to

A

Lynchsyndrome in patients with homozygous mu-tations of DNA mismatch repair genes

91
Q

%adults over 40dermatomyositis

mayhave an underlying malignancy, including agastrointestinal malignancy

A

15%-40%

92
Q

Subtype tylosis occurs between 5 and 15years of age and is associated with a high incidence esophageal carcinomas
(Howel Evans syn-drome).

A

Type A

93
Q

Paget cell histo

A

is large rounded cell with a clear pale vacuolated cytoplasm and a large eccentric nucleus, giving asignet ring appearance Positive stainingfor periodic acideSchiff mucin is also noted.

94
Q

? the link between inflammatory bowel disease and epidermolysis bullosa acquisita.

A

Autoantibodies to the NC1 domain of collagen VII generated during
gastrointestinal inflammation

95
Q

increase in the risk of non- Hodgkin lymphoma in patients

diagnosed with dermatitis herpetiformis ?

A

Did not show a statisticallysignificant when compared to matched controls.

96
Q

Which IBD lesions contiguous

with external mucous branes oral and perianal)

A

CD

97
Q

The severity of metastatic Crohn’s correlate with the severity
of the underlying Crohn’s disease?

A

does not always correlate Treat-ment of the latter does not necessarily guar-antee resolution of the former

98
Q

Pyoderma gangrenosum incidence in IBD?

A

shows a higher cor-relation to ulcerative colitis than to Crohn’sdisease

99
Q

In the setting of inflammatory bowel disease, which first-line treatment for refractory widespread pyoderma gangrenosum

A

tumor necrosis factorealfa inhibitors are considered

100
Q

EBA link with which IBD—

A

inparticular CD, with 25 cases in the literature—and rarely with UC, with only 4 cases

101
Q

bowel-associateddermatosis-arthritis syndrome

A

jejuonoileal bypass> conventional bariatric

intervention > laparoscopic gastric bypass

102
Q

Thelikely epitope responsible for immune
bowel-associated syndrome complex
formation in dermatosis-arthritis

A

Peptidoglycan

103
Q

Incidence of HenocheSch€on-lein purpura associated with group Abeta-hemolytic Streptococcus infections

A

Only a minority of cases

104
Q

Morphology of adult HenocheSch€onlein purpura cases

A

Bullous lesions are seen

105
Q

What systemic manifestations of HenocheSchonleinpurpura responsive to glucocorticoid administration,

A

gastrointestinal, rheumatologic , skin manifestations and theprogression of nephritis to end stage renaldisease are unaffected

106
Q

Whicg subclass of immunoglobulins responsible for the pathologic changes of HenocheSchonlein purpura

A

immunoglobulin A1

107
Q

DH- time taken for improvement -skin, GIT, from Institution of a gluten-free diet

A

rapidimprovement of the gastrointestinal symp-toms of dermatitis herpetiformis, while skinmanifestations may take up to 2 years toresolve

108
Q

Dermatitis herpetiformis is almost universally associated with human leukocyte, which are located on chromosome ?

A

DQ2 or DQ8, both of which are located on chromosome 6

109
Q

Severity of villous atrophy of duodenum in patients with dermatitisherpetiformis compared to those with celiacdisease

A

villous atrophy of duodenum

Is Generally milder in patients with dermatitisherpetiformis compared to those with celiacdisease

110
Q

increased risk of malignancy, includingnon-Hodgkin lymphoma, in the dermatitisherpetiformis population?

A

No

111
Q

Other tests for DH associations

A

Antithyroid peroxidase, thyroid-stimulating hormone, antigastric parietalcells, ANA, and anti-Ro
autoimmune disease

112
Q

pathophysiology of Degos disease

A

Dysregulation of interferon-alfa

membranolytic attack complex

113
Q

Successful treatment with eculizumab, monoclonal antibody to complement protein C5, for?

A

Degos disease

114
Q

GIT of HermanskyPudlak syndrome

A

granulomatous colitis , clinically like ulcerative colitis but is histologically more similar to Crohn’s disease

115
Q

Which agent has been successful in the treat-ment of the colitis of HermanskyePudlaksyndrome

A

Infliximab

116
Q

BLOC components and adaptor protein 3 involved in

lysosomal functioning defective in

A

HermanskyePudlak syndrome

117
Q

GIT management BRBN

A

Iron supplementation,
transfusion, endoscopic
coagulation, and resection(level IV)

118
Q

EDS subtype in which gastrointestinal complications are a prominent feature, occurring in 19% of these patients

A

Type IV vascular type EhlerseDanlos syndrome

119
Q

mutation in COL3A1 on chromosome 2q31 associated with

A

the vascular type EhlersDanlos syndrome

120
Q

Classical (EDS I and II)

Abnormal type ? collagen

A

type V

121
Q

Which EDs subtype Skin laxity, velvet skin, joint

hypermobility, and recurrentjoint dislocations

A

Type 3 hyper mobility

122
Q

Which EDS has Abnormal type III collagen and present
Arterial rupture, easy bruising,
hypermobility of small joints,and varicose veins

A

Vascular (EDS IV)

123
Q

Which EDS caused by AR, Deficiency of collagen lysyl hydroxylase-1.

A

Kyphoscoliosis (EDS VI)

124
Q

Which EDS subtype Deficiency of chains in type I collagen

A

Arthroclasia type VII

125
Q

Which EDS subtype Deficiency of enzyme in type I collagen

A

.Dermatosparaxis

126
Q

Sagging redundant skin, soft,
doughy skin texture, easy
bruising, hernias, and prematurerupture of fetal membranes

A

Dermatosparaxis

127
Q

pathognomonic ocular lesion for PXE

A

comet lesions,’’multiple small chorioretinal atrophies, Angioid streaks are not necessarily specific for PXE;

128
Q

Kaposi sarcoma staining for

A

D2-40
LYVE-1
Lymphatic Endothelial cell origin

129
Q

Casal’s necklace due to which vitamin deficiency

A

B3

130
Q

Treatment for pellagra

A

500 mg nicotinamide or

nicotinic acid a day forseveral weeks

131
Q

defective Zip4 transporter causes?

A

acrodermatitis entero-pathica

132
Q

When a diagnosis of acrodermatitis entero-pathica is suspected, investigations?

A

plasma zinc levels ,alkaline phosphatase level , which is a zinc-dependent enzyme and increases the sensitivity of detection

133
Q

fillers: foreign body granuloma, with numerous multinucleated
giant cells around translucent particles of different sizes,
most of them showing a fusiform or oval shape

A

Poly-L-lactic acid

134
Q

which filler is birefringent under polarized light examination.

A

Poly-L-lactic acid

135
Q

fillers: foreign body reaction around

many bluish round or oval particles packed together

A

Calcium hydroxylapatite

136
Q

fillers: well circumscribed palisading granulomas.

A

Bovine collagen

137
Q

which filler histopathologically,
granulomatous foreign body reaction, with abundant multinucleated giant cells surrounding an extracellular basophilic amorphous material,

A

hyaluronic acid gel

138
Q

resorbable filler that induces tissue augmentation that lasts up to
at least 24 months

A

Poly-L-lactic acid

139
Q

which filler microspheres stimulate almost no foreign body reaction and they appear bluish in color and round or oval in shape

A

CALCIUM HYDROXYLAPATITE

140
Q

consists of recurrent lower extremity purpura, an elevated erythrocyte sedimen-tation rate ESR and polyclonal gammopathy.

A

Hypergammaglobulinemic purpura of Waldenstr€om (HGPW)

141
Q

HGPW common serologic association,

A

Anti-Ro
antibodies
(SSA)

142
Q

Underlying associated with this syndrome Hypergammaglobulinemic Waldenstr€om (HGPW)

A

Sj€ogren syndrome

143
Q

pregnant patient with this condition HGPW would increased risk for

A

Intrauterine growth restriction,

fetal heartblock, and fetal demise

144
Q

HGPW Rx

A

Compression stockings, hydroxychloroquine, indomethacin

145
Q

MTS is distinguished by the development of

A

at least 1 cutaneous sebaceousneoplasm (sebaceous adenoma, sebaceous epithe-lioma, sebaceous carcinoma) or multiple keratoacanthomas, at least 1 visceral neoplasm

146
Q

Patients with MTS are at highest risk of what cancer

A

colorectal cancer, breast, genitourinary, neoplasms

147
Q

oral physiologic pigmentation typically affect

A

the attached gingiva withrelative sparing of the free gingiva.

148
Q

PJS malignant transformation of the polyps occurs in

A

2% to 3% ofcases.

149
Q

extraintestinal malignancies of PJS

A

The Breast , testicles, and ovaries

150
Q

diagnosis of exclusion and needs to be differentiated from other, more malignant systemic disorders, such as PJS.
Oral Pigmentation with or without pigmentation of thefingernails. The oral lesions present as black to brown macules commonly found on the hard palate,tongue, lips, and buccal mucosa. Associated mela-nonychia of the fingernails is observed in 50%

A

LaugiereHunziker syndrome

LHS

151
Q

Antimalarial drugs
Minocycline
Ketoconazole
Zidovudine to cause

A

oral melanosis

152
Q

pathogenesis

underlying physiologic hyperpigmentation ?

A

increase in number of melanocytesb. An increase in activity of melanocytes

153
Q

pathophysiology of HISN

A

type III hypersensitivity reaction in which antigen antibody complexes are depos-ited in the dermal blood vessels. Immune complex activation and aggregationof platelets

154
Q

Which method of assessment is used to estab-lish this diagnosis HISN

A

Clinical +_ skin biopsy specimen

155
Q

HISN RX

A

Supportive treatment and discontinuation ofthe offending medication

156
Q

classic skin finding in systemic amyloidosis ?

A

Pinch purpura

157
Q

median survival time for patientswith light chain amyloidosis?

A

6 to 15 months

158
Q

cylindromas

malignant transformation

A

cylindrocarcinoma.

159
Q

Unlike other forms of inflammatory arthritis, the arthropathy of MRH canbe identified by its characteristic joint space widening without

A

periosteal bone growth.

160
Q

MRH .Immunohistochemical analysis

A

monocyte/macrophage origin
positive CD45 (leukocyte common antigen),CD68
negative for S-100

161
Q

MRH major histocompatibility complex classII

A

human leukocyte antigen DR-1.

162
Q

Multiple Angiofibromas , found in

A

tuberous sclerosus complex(TSC) MEN1.

163
Q

Neutrophilic dermatosis of the dorsal hands (NDDH) test

A

Complete blood cell count—neutrophilia and leukocytosis, strong association with leukemia and myelodysplasia

164
Q

First-line therapies for NDDH, Sweet syndrome, and atypical pyoderma gangrenosum

A

include antineutrophilic agents, such as systemic corticosteroids, topical and intralesional corticosteroids, colchicine, and low dose dapsone. cyclosporine, methotrexate, and potassium iodide.

AAD ETAS (12 decks)

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