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
what is dress
drug reaction with eosinophilia and systemic symptoms | - rash and systemic upset incorporating haematological and solid organ disturbances
26
how is DRESS diagnosed
``` 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
what are the organs involved in DRESS
``` liver ( hepatitis) - most frequent cause of death kidneys - interstitial nephritis heart - myocarditis brain thyroid - thyroiditis lungs - interstitial pneumonitis ```
28
what are some meds that can cause DRESS
sulfonamides anti epileptics (carbamazepine, phentoin, lamotrigine) allopurinol, antibiotics( vancomycin, amoxicillin, minocycline, piperacillin - tazobactam), ibuprofen
29
what are the rash morphologies seen in DRESS
``` urticated papular exanthem - widespread papules maculopapular (morbilliform) eruption erythroderma head/neck oedema erythema multiforme- like ```
30
what is the treatment for DRESS
withdrawal of culprit corticosteroids = first line treatments, may require them for months mortality - 5-10%
31
what is graft versus host disease
multi organ disease can affect upto 80% of allogenic haematopoetic stem cell transplants, hsct pathogenesis - donor derived t lymphocyte activity against immunocompromised recipient
32
what does graft versus host disease mainly affect
skin liver gi tract
33
what is pruritus
``` 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
what are some investigations for pruritus
``` fbc, ldh renal profile lfts ferritin cxr hiv/ hep abc test ```
35
what is scuvy
vit c deficiency spongy gingivae with bleeding and erosion petechiae, ecchymoses, follicular hyperkeratosis corkscrew hairs with perifollicular haemorrhage
36
what are some other nutritional deficiencies
kwashiorkor - protein def zinc deficiency vit b3 def
37
what are some systemic features of kwashiokors
``` hepatomegaly bacterial/ fungal infections diarrhoea loss of muscle mass oedema failure to thrive ```
38
what are some skin signs of kwashiokors
superficial dequamation large areas of erosion sparse, dry hair soft thin nails cheilitis
39
what is the importance of zincP
plays a role in 200 enzymes - regulation of lipid, protein, nucleic acid synthesis has roles in wound healing, antioxidants and psychological benefits
40
what does a genetic (slc39a4) or acquired (dietary) deficiency of zinc cause
triad of Dermatitis, Diarrhoea and Depression perioral, acral and perineal skin in particular is affected with scaly erosive erythema
41
what does vit b3 deficiency cause
``` 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
what is carcinoid syndrome
signifies metastases of malignant carcinoid tumour 5ht secretion flushing in 25% cases other symptoms: diarrhoea, bronchospasm, hypotension
43
what is stevens- johnson syndrome/ toxic epidermal necrolysis
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
what is reason behind sjsten
when less than 10% of epidermal detachment - sjs more than 30% - ten in between is sjsten
45
what is the cause of sjsten
cell mediated cytotoxic reaction against epidermal cells drugs cause >80% of cases may be started upto 3 wks prior to onset of rash
46
what can sjsten sometimes be mistaken as
ssss- staph scalded skin syndrome thermal burns cutaneous graft versus host disease
47
whats used to diagnose sjsten
``` SCORTEN - score used to help assess severity age >40 hr initial % of epidermal detachment serum urea and glucose and bicarbonate presence of malignancy ```
48
what are some complications of sjsten
``` death - overall mortality 30% blindness, dehydration, hypothermia/hyperthermia renal tubular necrosis eroded gi tract interstitial pneumonitis liver and heart failure ```
49
what is erythroderma
generalised erythema affecting >90% of body
50
what are the systemic manifestations that reflect impairment in skin function in erythroderma
``` peripheral edema tachycardia loss of fluid and proteins disturbances in thermoregulation risk of sepsis ```
51
what are the multiples etiologies of erythroderma
``` drug reactions cutaneous t cell lymphoma - sezary syndrome psoriasis atopic eczema idiopathic ```
52
how can we manage erythroderma
``` treat underlying cause hospitalisation if systemically unwell restore fluid and electrolyte balance, circulatory status and manage body temp emollients topical steroids? antibiotics? ```
53
what are some cutaneous signs of systemic disease | e.g ckd
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
other cutaneous signs of systemic disease | e.g chronic liver disese
``` muehrckes lines terrys nails jaundice spider telangietasia porphyria cutaneous tarda palmar clubbing ```
55
what is necrobiosis lipoidica
20-65% cases occur in setting of dm | plaques with red brown raised edge with yellow atrophic centre
56
how to treat necrobiosis lipoidica
topical/ intralesional steroids
57
what are some other cutaneous manifestations of dm
``` terrys nails granuloma annulare neuropathic ulcers ancanthosis nigricans xerosis xanthelesma and xanthomata ```
58
what are some other maifestation in endo disorders
pretibial myxodem hyperpigmentation acne cutis gyrata verticis
59
what can you see in patients with hiv
``` severe seborrhoeic dermatitis extensive viral warts norwegian scabies cmv ulceration eosinophilic folliculitis bacillary angiomatosis kaposi sarcoma itch gential/oral ulceration ```
60
what can you see in skin in gi disorders
``` associated with ibd: pyoderma gangrenosum orofacial granulomatosis panniculitis aphthous ulceration association with psoriasis, pemphigold associated with celiac: dermatitis herpetiformis ```
61
what is hhidradenitis suppuritiva
inflammed nodules, sterile abcess, sinus tracts, fistulae and hypertrophic scars favours intertriginous zones esp axillary, anogenital and inframammary area
62
what is pyoderma gangrenosum
pustule on erythematous base - ulcerates and extends with necrotic undermined disorder painful associated with ibd, leukaemia, seronegative arthiritis
63
cutaneous signs of internal malignancy
cutaneous metastases malignancy reflecting internal malignancy - e.g pagets genetic condition skin disease asscoiated with malignancies non specific signs