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brs (phase 1b) > (derm) the skin in systemic disease > Flashcards

(derm) the skin in systemic disease Flashcards

1
Q

why is the skin important in systemic disease?

A

recognition of skin signs can lead to diagnosis of underlying systemic disease

  • allows recognition of internal malignancy
  • prevents or reduced internal organ damage when diagnosed early
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2
Q

what are three ways in which the skin can indicate systemic disease?

A

1) skin can be targeted in a multi-organs systemic disease (e.g. sarcoidosis)
2) tell-tale skin signs can indicate internal disorder (flushing in Carcinoid syndrome, pyoderma gangrenosum in IBD)
3) systemic disease secondary to skin disorder (e.g. cardiac failure due to erythroderma)

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3
Q

which systemic disease does pyoderma gangrenosum suggest?

A

inflammatory bowel disease

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4
Q

which blood tests are commonly done in dermatology?

A

full blood count

renal profile

liver function tests

inflammatory markers

autoimmune serology

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5
Q

which microbiology tests are commonly done in dermatology?

A

viral/bacterial serology

swabs for bacteria culture & sensitivities, viral PCR

tissue culture/PCR

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6
Q

which specialist tests are commonly done in dermatology?

A

skin biopsy
imaging
microscopy

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7
Q

what is a punch biopsy?

A

taking a punch-size piece of skin from the body

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8
Q

when are punch biopsies taken?

A

to study suspected melanomas, invasive carcinomas, inflammatory lesions and neoplasias

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9
Q

how are punch biopsies studied?

A

identify inflammatory patterns and cellular abnormalities

to detect autoantibodies in immunofluorescence

skin biopsy, tissue culture

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10
Q

what are the two main types of lupus erythematosus?

A

systemic lupus erythematosus = SLE

chronic cutaneous (discoid) lupus erythematosus = DLE

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11
Q

define pancytopenia

A

deficiency of all three cellular components of the blood (red cells, white cells, and platelets)

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12
Q

what are the musculocutaneous findings in lupus erythematosus?

A

chillblains

photo-distributed (sun-exposed areas) erythematous rash

alopecia

oral ulcers

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13
Q

what are the systemic findings in lupus erythematosis?

A

synovitis

serositis (pleurisy/pericarditis)

renal disorder

neurological disorder

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14
Q

what are the haematological findings in lupus erythematosis?

A

haemolytic anaemia
thrombocytopenia
leukopenia

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15
Q

what are the immunological findings in lupus erythematosis?

A

ANA
anti-dsDNA
anti-Sm
antiphospholipid

low complement

positive direct Coomb’s test

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16
Q

what are the cutaneous manifestations of systemic lupus erythematosus?

A

photo-distributed rash

chillblains

alopecia

cutaneous vasculitis (w palpable purpura) = purple spots/rashes bc of internal bleeding

livedo reticularis

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17
Q

what are the signs of discoid lupus erythematosus?

A

limited to cutaneous effect only, will not affect other organ systems SO

  • scarring (most prominent feature)
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18
Q

what do annular ring-like plaques suggest in a patient with lupus?

A

sign of subacute cutaneous lupus erythematosus

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19
Q

what is livedo reticularis?

A

net-like pattern of reddish-blue discolouration that

= indicates SLE

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20
Q

what is palpable purpura?

A

rash of purple spots on the skin due to internal bleeding from small blood vessels

= sign of SLE

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21
Q

a newborn presents with a ring-like annular rash

what is the most likely underlying disorder?

A

annular ring-like plaques in newborns indicate neonatal lupus

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22
Q

a newborn presents with a ring-like annular rash

what test must you do and why?

A

must do an ECG

= >50% risk that newborn has heart block and so urgently requires a pacemaker

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23
Q

what is dermatomyositis?

A

autoimmune connective tissue disease that causes muscle inflammation and skin rash

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24
Q

what are the two main features of dermatomyositis?

A

1) inflammatory myopathy

2) photo-distributed pink-violet rash

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25
Q

the muscles of which region are affected most commonly in dermatomyositis?

A

proximal extensors

= affected most by inflammatory myopathy

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26
Q

the skin of which region are affected most commonly in dermatomyositis?

A

scalp, periocular region, extensor surfaces

= affected most by photo-distributed skin rash

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27
Q

what are the key skin signs that are pathognomonic dermatomyositis?

A

Gottron’s papules

Shawl’s sign

ragged cuticles

heliotrope rash

photosensitive erythema

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28
Q

what are Gottron’s papules?

A

violaceous plaques on the MCP and distal ICP joints

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29
Q

what is Shawl’s sign?

A

redness of the upper back/trunk

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30
Q

what is a heliotrope rash?

A

violet, bluish-purple rash that develops on the skin

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31
Q

how are the subtypes of dermatomyositis differentiated?

A

different autoantibodies

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32
Q

what do anti Jo-1 antibodies in dermatomyositis suggest?

A

fever, myositis, Gottron’s papules

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33
Q

what do anti SRP antibodies in dermatomyositis suggest?

A

necrotising myopathy

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34
Q

what do anti p-155 antibodies in dermatomyositis suggest?

A

associated with malignancy in adults

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35
Q

which autoantibody would be associated with calcinosis in dermatomyositis?

A

anti p-140 antibody

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36
Q

why autoantibody is associated with amyopathic dermatomyositis?

A

anti SAE antibody

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37
Q

why autoantibody is associated with interstitial lung disease, digital ulcers and ischaemia in dermatomyositis?

A

anti MDA5 antibody

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38
Q

what do anti Mi-2 antibodies in dermatomyositis suggest?

A

mild muscle disease

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39
Q

which investigations are carried out for a patient with suspected dermatomyositis?

A
  • ANA
  • autoantibody panel for DMS
  • LFT (as ALT is often increased)
  • CK
  • EMG (electromyography)
  • skin biopsy
  • screening for internal malignancy
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40
Q

what is IgA vasculitis?

A

(Henoch-Schonlein purpura)

when IgA antibodies collect in small blood vessels, which then become inflamed and leak blood

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41
Q

what are the symptoms of IgA vasculitis?

A

abdominal pain
gastrointestinal bleeding

arthralgia
arthritis

IgA-associated glomerulonephritis

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42
Q

why do the symptoms of IgA vasculitis occur?

A

vasculitis usually affect the small blood vessels of the GI tract

= abdominal pain, GI bleeding etc

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43
Q

which combination/types of blood vessels are commonly affected by vasculitis?

A

small
small & medium
medium
large

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44
Q

in which conditions are small vessels affected by vasculitis?

A

cutaneous small-vessel vasculitis

  • (idiopathic, infectious, inflammatory, drug exposure)
  • IgA vasculitis
  • urticarial vasculitis
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45
Q

in which conditions are small & medium vessels affected by vasculitis?

A

ANCA-associated vasculitis

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46
Q

in which conditions are medium vessels affected by vasculitis?

A

polyarteritis nodosa (PAN)

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47
Q

in which conditions are large vessels affected by vasculitis?

A

temporal arteritis

tayakasu’s arteritis

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48
Q

what type of vasculitis do macular/palpable purpura indicate?

A

small vessel vasculitis

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49
Q

what are the main manifestations of medium vessel vasculitis?

A

digital necrosis

retiform purpura ulcers

subcutaneous nodules along blood vessels

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50
Q

differentiate between macular and palpable purpura

A 
macular = flat
palpable = raised
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51
Q

what is digital necrosis a sign of?

A

necrosis of the digits

= medium vessel vasculitis

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52
Q

what are retiform purpura ulcers a sign of?

A

medium vessel vasculitis

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53
Q

what are subcutaneous nodules along blood vessels a sign of?

A

medium vessel vasculitis

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54
Q

when can you get saddle-node deformity?

A

ANCA-associated vasculitis

= if granulomatous, needs to be treated aggressively as it can be fatal

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55
Q

what is sarcoidosis?

A

systemic granulomatous disorder of unknown origin with variable presentations

affects many organ systems, but mainly the lungs

can also have cutaneous manifestations

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56
Q

what are the cutaneous manifestations of sarcoidosis?

A

red-brown to violaceous papules and face, lips, upper back, neck, and extremities

lupus pernio

ulcerative

scar sarcoid

erythema nodosum

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57
Q

what is lupus pernio?

A

chronic raised indurated (hardened) lesion of the skin

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58
Q

what is erythema nodosum?

A

swollen fat under the skin causing red bumps and patches

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59
Q

what is the histology result for sarcoidosis?

A

non-caseating epithelioid granulomas

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60
Q

what must you do in a patient with sarcoidosis?

A

1) diagnosis of exclusion

2) requires evaluation of internal organ involvement

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61
Q

what is the most common skin sign of sarcoidosis?

A

granulomatous plaques

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62
Q

what is DRESS?

A

Drug Reaction with Eosinophilia and Systemic Symptoms

= severe hypersensitivity reaction to a drug that causes rash & systemic disturbance incorporating haematological and solid-organ disturbances

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63
Q

what is the diagnostic criteria for DRESS?

A

fever features:

  • fever ≥ 38.5°C
  • lymphadenopathy ⩾ 2 sites, > 1cm

blood features:

  • circulating atypical lymphocytes
  • peripheral hypereosinophilia > 0.7 × 109
  • negative ANA & bacterial/viral cultures

involvement features:

  • internal organs involvement (liver, kidneys, cardiac)
  • skin involvement
64
Q

what is the extent of skin involvement required for a diagnosis of DRESS?

A

1) > 50% BSA (body surface area)
2) cutaneous eruption suggestive of DRESS e.g. facial oedema
3) biopsy suggestive of DRES

65
Q

which internal organs are involved in DRESS?

A

liver (mos frequent)

kidney (interstitial nephritis)

heart (myocarditis)

brain

thyroid (thyroiditis)

lung (interstitial pneumonitis)

66
Q

which internal organ is the most frequent cause of death in DRESS?

A

liver

67
Q

when do symptoms of DRESS first start to appear?

A

approx 2-6 week following drug exposure

68
Q

which drugs are common triggers for DRESS?

A 
sulfonamides
antibiotics
allopurinol
anti-epileptics
ibuprofen
69
Q

what are the skin signs of DRESS?

A

neck/head oedema

erythema multiforme-like rash

erythroderma

maculopapular eruption

urticated papular exanthem

70
Q

what is an erythema multiforme-like rash?

A

raised, red, target-like rash on the skin or mucous membranes

71
Q

what is the treatment for DRESS?

A

withdraw culprit drug

administer corticosteroids

72
Q

how can you tell if a rash is caused by an adverse drug reaction OR by graft versus host disease?

A

face involvement
acral involvement (limbs)
diarrhoea

= all indicate that GvHD is more likely than a rash

73
Q

what is graft versus host disease?

A

a complication of an allogeneic stem cell transplantation wherein the donor T lymphocytes mount an immune response against the host cells

74
Q

briefly explain the pathogenesis of GvHD

A

donor-derived T-lymphocytes (graft) mount an inappropriate immune response against antigens on host cells

75
Q

which group of people are affected most by GvHD?

A

approx 10-80% of allogenic haematopoetic stem cell transplant patients

76
Q

which parts of the body are affected most commonly by GvHD?

A

skin
liver
GI tracts

77
Q

what is graft versus host disease?

A

a complication of an allogeneic stem cell transplantation wherein the donor T lymphocytes mount an immune response against the host cells

78
Q

briefly explain the pathogenesis of GvHD

A

donor-derived T-lymphocytes (graft) mount an inappropriate immune response against antigens on host cells

79
Q

which group of people are affected most by GvHD?

A

approx 10-80% of allogenic haematopoetic stem cell transplant patients

80
Q

which parts of the body are affected most commonly by GvHD?

A

skin
liver
GI tract

81
Q

define pruritus

A

itching

82
Q

what does itching without a rash suggest?

A

suggestive of an internal cause

83
Q

what are the possible causes of pruritus without a rash?

A

haemotological causes:

  • polycythemia
  • lymphoma
  • iron deficiency/overload

diseases:

  • HIV
  • hepatitis A/B/C
  • uraemia
  • cholestasis
  • cancer

pharmacological:
- drugs (opiates/opioids)

miscellaneous:

  • pruritus of old age
  • psychogenic
84
Q

which investigations are carried out for pruritus?

A

FBC
liver function tests
renal profile

ferritin, LDH

test for HIV
test for hepatitis A/B/C

CXR

85
Q

what can pruritus lead to?

A

nodular prurigo

86
Q

what is nodular prurigo?

A

skin thickening in the form of large lumps due to excessive itching

87
Q

what is scurvy?

A

vitamin C (ascorbic acid) deficiency

88
Q

what are the systemic features of Kwashiorkor?

A 
hepatomegaly
bacterial/fungal infections
oedema
diarrhoea
loss of muscle mass
89
Q

what are the cutaneous manifestations of Kwashiorkor?

A

superficial desquamation
thin nails
sparse hair
chelitis (inflammation of the lips)

90
Q

which nutritional deficiency are corkscrew hairs indicative of?

A

scurvy (vitamin C deficiency)

91
Q

why is zinc important in the body?

A

required for the functioning of over 200 enzymes (lipid, nucleic acid synthesis)

would healing

antioxidant properties

92
Q

which triad of symptoms is present in zinc deficiency?

A

Depression
Diarrhoea
Dermatitis

93
Q

what are the cutaneous manifestations of zinc deficiency?

A

perineal, perioral and acral scaly erosive erythema

94
Q

which tetrad of symptoms is present in niacin deficiency?

A

Diarrhoea
Dermatitis
Dementia
Death

95
Q

what is niacin also known as?

A

vitamin B3

96
Q

what are the cutaneous manifestations of niacin deficiency?

A

photodistributed erythema

Casal’s necklace

painful fissures of the palm and soles

perianal, perioral and genital inflammation and erosion

97
Q

which nutritional deficiency is superficial desquamation indicative of?

A

kwashiorkor (protein deficiency)

98
Q

which nutritional deficiency is Casal’s necklace indicative of?

A

vitamin B3 (niacin) deficiency

99
Q

which nutritional deficiency is chelitis indicative of?

A

kwashiorkor (protein deficiency)

100
Q

what is carcinoid syndrome?

A

occurs when a carcinoid tumour metastasises and secreted 5-HT into the systemic circulation

101
Q

which molecule is secreted in large amounts in carcinoid syndrome?

A

5-HT

102
Q

what are the symptoms of carcinoid syndrome?

A

flushing
diarrhoea
brochospasm
hypotension

103
Q

what is SJS-TEN?

A

cell-mediated cytotoxic reaction against epidermal cells

104
Q

what symptoms do SJS-TEN patients have in the early stages?

A

flu-like symptoms

105
Q

how do the symptoms of an SJS-TEN patient develop?

A

flu-like symptoms

abrupt onset of lesions on trunk, face, limbs

macules, blisters, erythema

blisters merge

sheets of skin detachment

106
Q

what is the characteristic cutaneous manifestation of SJS-TEN?

A

epidermal necrolysis = sheets of skin detachment

107
Q

how long does it take for SJS-TEN patients to develop extensive full-thickness epidermal necrosis?

A

approx 2-3 days

108
Q

why does SJS-TEN refer to two conditions?

A 
one a continuum based on % BSA affected
i.e.
<10% affected = SJS
10-30% affected = SJS-TEN
>30% affected = TEN
109
Q

what constitutes the classification of SJS?

A

<10% BSA = SJS

110
Q

what constitutes the classification of TEN?

A

10-30% BSA = SJS-TEN

111
Q

what constitutes the classification of SJS-TEN?

A

> 30% BSA = TEN

112
Q

what is the mortality of SJS compared to TEN?

A

10%-

113
Q

which two prognostic scores are used for SJS-TEN?

A

30%+

114
Q

what causes SJS-TEN?

A

drugs cause >80% of cases

115
Q

which drugs can cause SJS-TEN?

A 
sulphonamides
antibiotics
allopurinol
anti-epileptics
NSAIDs (e.g. ibuprofen)

(same list as drugs that cause DRESS!)

116
Q

which conditions resemble SJS-TEN?

A

SSSS (staphylococcal scalded skin syndrome)

graft versus host disease

thermal burns

117
Q

what is the criteria for SCORTEN?

A

a prognostic tool used to assess severity of SJS-TEN

  • age > 40
  • HR
  • initial % epidermal detachment
  • presence of malignancy
  • serum urea, glucose, bicarb
118
Q

what are the complications for SJS-TEN?

A

mortality rate = >30% = death

blindness
dehydration
hypothermia/hyperthermia
renal tubular necrosis
eroded GI tract
interstitial pneumonitis, neutropenia
liver and heart failure
psychological (e.g. PTSD)
119
Q

what is erythroderma?

A

generalized erythema affecting >90% of BSA

120
Q

what are the systemic manifestations of erythroderma?

A

peripheral oedema

tachycardia

loss of fluid and proteins

disturbances in thermoregulation

risk of sepsis

121
Q

what causes erythroderma?

A

drug reactions

psoriasis

atopic eczema

idiopathic

cutaneous T-cell lymphoma = Sezary syndrome

(padic mnemonic)

122
Q

how is erythroderma treated?

A

treat underlying cause OR hospitalise

treat w emollients to support skin barrier

topical steroids, antibiotics

restore fluid and electrolyte balance, circulatory status and manage body temperature

123
Q

what are the cutaneous signs of chronic kidney disease?

A 
excoriations
prurigo
xerosis
half & half nails
calciphylaxis

(anaemia = mucosal pallor, hair thinning)

1) signs related to causal primary disease (ANCA vasculitis, SLE)
2) signs related to immunosuppression (viral warts)

124
Q

what are the cutaneous signs of chronic liver disease?

A 
Terry's nails
Muerhke's lines
jaundice
spider telangiectasia
palmar erythema
clubbing
porphyria cutanea tardia
125
Q

what is necrobiosis lipodica?

A

plaques with red-brown raised edge with yellow-brown atrophic centres

= indicative of diabetes mellitus

126
Q

what is the treatment for necrobiosis lipodica?

A

topical/intralesional steroids

127
Q

what are the cutaneous manifestations of diabetes mellitus?

A 
Terry's nails
xerosis
granuloma annulare
neuropathic ulcers
acanthosis nigricans
xanthelasma & xanthomata
skin infections
128
Q

which endocrinological disorder is indicated by pre-tibial myxoedema?

A

Graves’ disease

129
Q

which endocrinological disorder is indicated by hyperpigmentation?

A

Addison’s disease

130
Q

which endocrinological disorders are indicated by acne?

A

PCOS
acromegaly
Cushing’s

131
Q

which endocrinological disorder is indicated by cutis gyrata verticis?

A

acromegaly

132
Q

what are the cutaneous manifestations of acromegaly?

A

cutis gyrata verticis

acne

133
Q

what are the cutaneous manifestations of HIV?

A 
severe seborrhoeic dermatitis
eosinophilic folliculitis
extensive viral warts
CMV ulceration
severe psoriasis
bacillary angiomatosis
Norwegian scabies
Kaposi sarcoma
134
Q

what are the variable non-specific manifestations of HIV seroconversion?

A

urticaria
erythema multiforme
morbiliform rash
oral/geniral ulceration

= so, low threshold for testing

135
Q

what kind of infections are patients with HIV likely to contract?

A

opportunistic infections

atypical manifestations of common infections

severe forms of common dermatoses (severe seborrhoeic dermatitis, severe psoriasis)

suggestive dermatoses (eosinophilic folliculitis)

136
Q

what are the cutaneous manifestations of IBD?

A

pyoderma gangrenosum

orofacial granulomatosis

panniculitis (erythema nodosum)

aphthous ulceration

associated with psoriasis, pemphigoid

137
Q

what GI condition is dermatitis herpetiformis associated with?

A

coeliac disease

138
Q

what can hidradenitis suppurtiva suggest?

A

high BMI, diabetes mellitus OR inflammatory bowel disease

139
Q

describe the pathology of hidradenitis suppuritiva

A

inflamed nodes + abscesses rupture and cause extensive scarring
= pain, malodour, discharge

140
Q

which regions of the body are most commonly affected by hidradenitis suppuritiva?

A

intertriginous zones (zones w two skin areas rubbing against each other)

  • axillary, anogenital, inframammary
141
Q

what is pyoderma gangrenosum?

A

painful pustule on an erythematous base that ulcerates and has a necrotic border

142
Q

which conditions are associated with pyoderma gangrenosusm?

A

inflammatory bowel disease
seronegative arthritis
leukaemia

143
Q

which skin diseases are associated with malignancy?

A

dermamyositis
erythema gyratum repens
pyoderma gangrenosum
paraneoplastic pepmhigus

(depp)

144
Q

which genetic conditions predispose to internal cancer and

A

Peutz-Jeghers syndrome

hereditary leiomyomatosis

renal cell cancer

145
Q

which condition is indicated as a result of peau d’orange?

A

breast carcinoma

146
Q

which condition is indicated as a result of groin metastases?

A

prostatic carcinoma

147
Q

which condition is indicated as a result of leukaemia cutis?

A

leukaemia

148
Q

which condition is indicated as a result of haemorrhagic nodules?

A

metastatic pancreatic carcinoma

149
Q

which condition is indicated as a result of extramammary Paget’s disease?

A

renal/pelvic malignancy

150
Q

which condition is indicated as a result of acanthosis nigricans?

A

diabetes

151
Q

which condition is indicated as a result of Paget’s disease of the nipple?

A

breast carcinoma

152
Q

which condition is indicated as a result of erythema gyratum repens?

A

bronchial carcinoma

153
Q

which condition is indicated as a result of paraneoplastic pemphigus?

A

malignancy

154
Q

which condition is indicated as a result of mucosal melanosis?

A

Peutz-Jeghers syndrome (genetic)

155
Q

which condition is indicated as a result of leiomyomas?

A

hereditary leiomyomatosis with renal cell cancer

brs (phase 1b) (71 decks)

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