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Skin in systemic disease Flashcards

1
Q

why is the skin important in systemic disease?

A

rashes are more than skin deep

recognition of skin diseases can help diagnose underlying conditions and prevent and treat organ damage

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2
Q

what can skin tell us

A

multi organ systemic disease
sign of internal disorder
skin condition suggesting underlying disorder
systemic disease secondary to skin disorder

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3
Q

what are the different types of causes you can find with skin disease

A 
idiopathic
neoplastic
infection
inflammatory
drug induced
autoimmune
traumatic
metabolic
genetic
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4
Q

what are some blood tests that you can carry out to look out for skin disease

A 
full blood count
renal profile
liver function tests
inflammatory markers
autoimmune serology
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5
Q

what are some microbiology that can be carried out to look for skin disease

A

viral/bacterial serology
swabs for bacteria c&s ,viral pcr
tissue culture/pcr

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6
Q

what is some imaging investigations that can be done for skin disease

A

internal organ involvement

vascular supply

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7
Q

what can you use to examine a skin biopsy

A

microscopy - cells are examined for abnormalities

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8
Q

what are some specific tests you could carry out to look for skin disease

A

urinalysis
nerve conduction studies
endocrine investigations etcc

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9
Q

what are the 2 types of lupus erythematosus

A

systemic lupus erythematosus
cutaneous (discoid) lupus eryhtematosus
is overlap between them

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10
Q

what is the diagnostic criteria for sle

A 
photodistributed rash - acute lupus
oral ulcers
alopecia
synovitis
serositis
renal disorder
neurological disorder
haematological findings: haemolytic anaemia
thrombocytopenia
leukopenia
And immunological findings
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11
Q

what are the immunological findings for sle

A 
ana
anti ds-dna
anti sm
antiphospholipid
low complement
direct coombs test
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12
Q

what are some other signs of sle

A

livedo reticularis - discolouration
palpable purpura- small vessel cutaneous vasculitis
subacute cutaneous lupus - chronic skin pointing ( also found in dle)

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13
Q

what is the distinctive feature of dle

A

scarring

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14
Q

what is the test for neonatal lupus

A

ecg - 50% of neonates have heart block

r0 postive

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15
Q

what is dermatomyotitis

A

autoimmune connective tissue disease

coupled with extensor inflammatory myopathy

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16
Q

what do we see with dermomyotitis

A 
photo distributed pink violet rash favouring scalp, periocular regions and extensor surfaces 
ragged cuticles
shawl sign - redness on upper trunk
heliptrope rash
gottrons papules
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17
Q

what is gottrons papules

A

veracious plaques in metacarpal phalangeal joints and distal interphalangeal joints
seen in dermamyotitis

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18
Q

what are certain subtypes of dermamyositis associated with

A

different antibodies

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19
Q

what are the investigations for dermatomyositis

A 
anti nuclear antibodies
ck
skin biopsy
lfts
emg
screening for internal malignnacy
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20
Q

what is iga vasculitis

A

type of vasculitis affecting small blood vessels in gi tract - causes abdominal pain and gi bleeding
affects joints and can affect kidneys

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21
Q

what are some subclassifications of of small vessel vascultitis - special types

A

iga vasculitis, utricarial vasculitis, acute haemorrhagic oedema of infancy and erythema elavatum diutinum

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22
Q

clinical vasculitis clinical features

A

small vessel - palpable/macular purpura

medium - digital necrosis, retiform purpura, ulcers, subcutaneous nodules along blood vessels

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23
Q

what do you see in anca associated vasculitis

A

saddle nose deformity
ulcerative rash
granulomatous

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24
Q

what is sarcoidosis

A

systemic granulomatosus disorder of unknown origin
can affect multiple organs - most commonly lungs
cutaneous manifestations - 33%
highly variable
non caseating epithelioid granulomas
disgnosis of exclusion

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25
Q

what is dress

A

drug reaction with eosinophilia and systemic symptoms

- rash and systemic upset incorporating haematological and solid organ disturbances

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26
Q

how is DRESS diagnosed

A 
based on scoring criteria including:
fever >38.5
lymphadenopathy >2 sites, >1cm
circulating atypical lymphocytes
peripheral hyperesosinophilia
internal organ involvement
negative ana, hep
skin involvement
biopsy suggesting DRESS
27
Q

what are the organs involved in DRESS

A 
liver ( hepatitis) - most frequent cause of death
kidneys - interstitial nephritis
heart - myocarditis
brain
thyroid - thyroiditis
lungs - interstitial pneumonitis
28
Q

what are some meds that can cause DRESS

A

sulfonamides
anti epileptics (carbamazepine, phentoin, lamotrigine)
allopurinol,
antibiotics( vancomycin, amoxicillin, minocycline, piperacillin - tazobactam), ibuprofen

29
Q

what are the rash morphologies seen in DRESS

A 
urticated papular exanthem - widespread papules
maculopapular (morbilliform) eruption
erythroderma
head/neck oedema
erythema multiforme- like
30
Q

what is the treatment for DRESS

A

withdrawal of culprit
corticosteroids = first line treatments, may require them for months
mortality - 5-10%

31
Q

what is graft versus host disease

A

multi organ disease
can affect upto 80% of allogenic haematopoetic stem cell transplants, hsct
pathogenesis - donor derived t lymphocyte activity against immunocompromised recipient

32
Q

what does graft versus host disease mainly affect

A

skin
liver
gi tract

33
Q

what is pruritus

A 
itching without rash - suggestive of internal cause:
- haematological: lymphoma, polycythaemia
- uraemia
- cholestasis
iron def/overload
hiv/hep abc
cancer
drugs
psychogenic
old age
look for nodular purigo
34
Q

what are some investigations for pruritus

A 
fbc, ldh
renal profile
lfts
ferritin
cxr
hiv/ hep abc test
35
Q

what is scuvy

A

vit c deficiency
spongy gingivae with bleeding and erosion
petechiae, ecchymoses, follicular hyperkeratosis
corkscrew hairs with perifollicular haemorrhage

36
Q

what are some other nutritional deficiencies

A

kwashiorkor - protein def
zinc deficiency
vit b3 def

37
Q

what are some systemic features of kwashiokors

A 
hepatomegaly
bacterial/ fungal infections
diarrhoea
loss of muscle mass
oedema 
failure to thrive
38
Q

what are some skin signs of kwashiokors

A

superficial dequamation large areas of erosion
sparse, dry hair
soft thin nails
cheilitis

39
Q

what is the importance of zincP

A

plays a role in 200 enzymes - regulation of lipid, protein, nucleic acid synthesis
has roles in wound healing, antioxidants and psychological benefits

40
Q

what does a genetic (slc39a4) or acquired (dietary) deficiency of zinc cause

A

triad of Dermatitis, Diarrhoea and Depression

perioral, acral and perineal skin in particular is affected with scaly erosive erythema

41
Q

what does vit b3 deficiency cause

A 
dermatitis, diarrhoea dementia and death
cutaneous manifestations:
- photodistributed erythema
casals necklace
painful fissures of palms and soles
peri-anal, genital and perioral inflammation and erosions
42
Q

what is carcinoid syndrome

A

signifies metastases of malignant carcinoid tumour
5ht secretion
flushing in 25% cases
other symptoms: diarrhoea, bronchospasm, hypotension

43
Q

what is stevens- johnson syndrome/ toxic epidermal necrolysis

A

derm emergency!
flu like sick
abrupt onset of lesions on trunk, then face and limbs,
macules, blisters, erythema
blisters merge
extensive full thickness mucocutaneous (epidermal) necrosis <2-3days

44
Q

what is reason behind sjsten

A

when less than 10% of epidermal detachment - sjs
more than 30% - ten
in between is sjsten

45
Q

what is the cause of sjsten

A

cell mediated cytotoxic reaction against epidermal cells
drugs cause >80% of cases
may be started upto 3 wks prior to onset of rash

46
Q

what can sjsten sometimes be mistaken as

A

ssss- staph scalded skin syndrome
thermal burns
cutaneous graft versus host disease

47
Q

whats used to diagnose sjsten

A 
SCORTEN - score used to help assess severity 
age >40
hr
initial % of epidermal detachment
serum urea and glucose and bicarbonate
presence of malignancy
48
Q

what are some complications of sjsten

A 
death - overall mortality 30%
blindness, dehydration, hypothermia/hyperthermia
renal tubular necrosis
eroded gi tract
interstitial pneumonitis
liver and heart failure
49
Q

what is erythroderma

A

generalised erythema affecting >90% of body

50
Q

what are the systemic manifestations that reflect impairment in skin function in erythroderma

A 
peripheral edema
tachycardia
loss of fluid and proteins
disturbances in thermoregulation
risk of sepsis
51
Q

what are the multiples etiologies of erythroderma

A 
drug reactions
cutaneous t cell lymphoma - sezary syndrome
psoriasis
atopic eczema
idiopathic
52
Q

how can we manage erythroderma

A 
treat underlying cause 
hospitalisation if systemically unwell
restore fluid and electrolyte balance, circulatory status and manage body temp
emollients
topical steroids?
antibiotics?
53
Q

what are some cutaneous signs of systemic disease

e.g ckd

A

excoriations/ prurigo
xerosis
half and half nails
calciphylaxis
and
sign related to primary disease - vasculitis, sle
signs related to immunosupression - viral warts, skin cancer

54
Q

other cutaneous signs of systemic disease

e.g chronic liver disese

A 
muehrckes lines
terrys nails
jaundice
spider telangietasia
porphyria cutaneous tarda
palmar
clubbing
55
Q

what is necrobiosis lipoidica

A

20-65% cases occur in setting of dm

plaques with red brown raised edge with yellow atrophic centre

56
Q

how to treat necrobiosis lipoidica

A

topical/ intralesional steroids

57
Q

what are some other cutaneous manifestations of dm

A 
terrys nails
granuloma annulare
neuropathic ulcers
ancanthosis nigricans
xerosis
xanthelesma and xanthomata
58
Q

what are some other maifestation in endo disorders

A

pretibial myxodem
hyperpigmentation
acne
cutis gyrata verticis

59
Q

what can you see in patients with hiv

A 
severe seborrhoeic dermatitis
extensive viral warts
norwegian scabies
cmv ulceration
eosinophilic folliculitis
bacillary angiomatosis
kaposi sarcoma
itch
gential/oral ulceration
60
Q

what can you see in skin in gi disorders

A 
associated with ibd:
pyoderma gangrenosum
orofacial granulomatosis
panniculitis
aphthous ulceration
association with psoriasis, pemphigold
associated with celiac: dermatitis herpetiformis
61
Q

what is hhidradenitis suppuritiva

A

inflammed nodules, sterile abcess, sinus tracts, fistulae and hypertrophic scars
favours intertriginous zones esp axillary, anogenital and inframammary area

62
Q

what is pyoderma gangrenosum

A

pustule on erythematous base - ulcerates and extends with necrotic undermined disorder
painful
associated with ibd, leukaemia, seronegative arthiritis

63
Q

cutaneous signs of internal malignancy

A

cutaneous metastases
malignancy reflecting internal malignancy - e.g pagets
genetic condition
skin disease asscoiated with malignancies
non specific signs

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