Skin in systemic disease Flashcards
why is the skin important in systemic disease?
rashes are more than skin deep
recognition of skin diseases can help diagnose underlying conditions and prevent and treat organ damage
what can skin tell us
multi organ systemic disease
sign of internal disorder
skin condition suggesting underlying disorder
systemic disease secondary to skin disorder
what are the different types of causes you can find with skin disease
idiopathic neoplastic infection inflammatory drug induced autoimmune traumatic metabolic genetic
what are some blood tests that you can carry out to look out for skin disease
full blood count renal profile liver function tests inflammatory markers autoimmune serology
what are some microbiology that can be carried out to look for skin disease
viral/bacterial serology
swabs for bacteria c&s ,viral pcr
tissue culture/pcr
what is some imaging investigations that can be done for skin disease
internal organ involvement
vascular supply
what can you use to examine a skin biopsy
microscopy - cells are examined for abnormalities
what are some specific tests you could carry out to look for skin disease
urinalysis
nerve conduction studies
endocrine investigations etcc
what are the 2 types of lupus erythematosus
systemic lupus erythematosus
cutaneous (discoid) lupus eryhtematosus
is overlap between them
what is the diagnostic criteria for sle
photodistributed rash - acute lupus oral ulcers alopecia synovitis serositis renal disorder neurological disorder haematological findings: haemolytic anaemia thrombocytopenia leukopenia And immunological findings
what are the immunological findings for sle
ana anti ds-dna anti sm antiphospholipid low complement direct coombs test
what are some other signs of sle
livedo reticularis - discolouration
palpable purpura- small vessel cutaneous vasculitis
subacute cutaneous lupus - chronic skin pointing ( also found in dle)
what is the distinctive feature of dle
scarring
what is the test for neonatal lupus
ecg - 50% of neonates have heart block
r0 postive
what is dermatomyotitis
autoimmune connective tissue disease
coupled with extensor inflammatory myopathy
what do we see with dermomyotitis
photo distributed pink violet rash favouring scalp, periocular regions and extensor surfaces ragged cuticles shawl sign - redness on upper trunk heliptrope rash gottrons papules
what is gottrons papules
veracious plaques in metacarpal phalangeal joints and distal interphalangeal joints
seen in dermamyotitis
what are certain subtypes of dermamyositis associated with
different antibodies
what are the investigations for dermatomyositis
anti nuclear antibodies ck skin biopsy lfts emg screening for internal malignnacy
what is iga vasculitis
type of vasculitis affecting small blood vessels in gi tract - causes abdominal pain and gi bleeding
affects joints and can affect kidneys
what are some subclassifications of of small vessel vascultitis - special types
iga vasculitis, utricarial vasculitis, acute haemorrhagic oedema of infancy and erythema elavatum diutinum
clinical vasculitis clinical features
small vessel - palpable/macular purpura
medium - digital necrosis, retiform purpura, ulcers, subcutaneous nodules along blood vessels
what do you see in anca associated vasculitis
saddle nose deformity
ulcerative rash
granulomatous
what is sarcoidosis
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
what is dress
drug reaction with eosinophilia and systemic symptoms
- rash and systemic upset incorporating haematological and solid organ disturbances
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
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
what are some meds that can cause DRESS
sulfonamides
anti epileptics (carbamazepine, phentoin, lamotrigine)
allopurinol,
antibiotics( vancomycin, amoxicillin, minocycline, piperacillin - tazobactam), ibuprofen
what are the rash morphologies seen in DRESS
urticated papular exanthem - widespread papules maculopapular (morbilliform) eruption erythroderma head/neck oedema erythema multiforme- like
what is the treatment for DRESS
withdrawal of culprit
corticosteroids = first line treatments, may require them for months
mortality - 5-10%
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
what does graft versus host disease mainly affect
skin
liver
gi tract
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
what are some investigations for pruritus
fbc, ldh renal profile lfts ferritin cxr hiv/ hep abc test
what is scuvy
vit c deficiency
spongy gingivae with bleeding and erosion
petechiae, ecchymoses, follicular hyperkeratosis
corkscrew hairs with perifollicular haemorrhage
what are some other nutritional deficiencies
kwashiorkor - protein def
zinc deficiency
vit b3 def
what are some systemic features of kwashiokors
hepatomegaly bacterial/ fungal infections diarrhoea loss of muscle mass oedema failure to thrive
what are some skin signs of kwashiokors
superficial dequamation large areas of erosion
sparse, dry hair
soft thin nails
cheilitis
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
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
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
what is carcinoid syndrome
signifies metastases of malignant carcinoid tumour
5ht secretion
flushing in 25% cases
other symptoms: diarrhoea, bronchospasm, hypotension
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
what is reason behind sjsten
when less than 10% of epidermal detachment - sjs
more than 30% - ten
in between is sjsten
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
what can sjsten sometimes be mistaken as
ssss- staph scalded skin syndrome
thermal burns
cutaneous graft versus host disease
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
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
what is erythroderma
generalised erythema affecting >90% of body
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
what are the multiples etiologies of erythroderma
drug reactions cutaneous t cell lymphoma - sezary syndrome psoriasis atopic eczema idiopathic
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?
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
other cutaneous signs of systemic disease
e.g chronic liver disese
muehrckes lines terrys nails jaundice spider telangietasia porphyria cutaneous tarda palmar clubbing
what is necrobiosis lipoidica
20-65% cases occur in setting of dm
plaques with red brown raised edge with yellow atrophic centre
how to treat necrobiosis lipoidica
topical/ intralesional steroids
what are some other cutaneous manifestations of dm
terrys nails granuloma annulare neuropathic ulcers ancanthosis nigricans xerosis xanthelesma and xanthomata
what are some other maifestation in endo disorders
pretibial myxodem
hyperpigmentation
acne
cutis gyrata verticis
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
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
what is hhidradenitis suppuritiva
inflammed nodules, sterile abcess, sinus tracts, fistulae and hypertrophic scars
favours intertriginous zones esp axillary, anogenital and inframammary area
what is pyoderma gangrenosum
pustule on erythematous base - ulcerates and extends with necrotic undermined disorder
painful
associated with ibd, leukaemia, seronegative arthiritis
cutaneous signs of internal malignancy
cutaneous metastases
malignancy reflecting internal malignancy - e.g pagets
genetic condition
skin disease asscoiated with malignancies
non specific signs
