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dermatology yr 2 > the skin in systemic disease > Flashcards

the skin in systemic disease Flashcards

1
Q

why is it important to see how the skin is affected by systemic disease

A

because rashes may be more than skin deep:
Prevent or reduce internal organ damage by early diagnosis
Allow detection of internal malignancy

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2
Q

what can be diagnosed if theres a ring like or annular rash? why is that diagnosis crucial? what do you need to do after you see/ diagnose this?

A

neonatal lupus, which is important because there is a 50% risk of heart block. It is important to perform an ECG.

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3
Q

what are investigations that you should do when you see skin stuff/ consider differentials

A

blood tests: FBC, renal, LFT, Infl markers, autoimmune serology

microbiology: viral/ bacterial serology, PCR, cultures, swabs for bacteria and C&S

imaging for vascular stuff and internal organ check

skin biopsy: microscopy

specific stuff: urinalysis, nerve conduction, endocrine investig

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4
Q

what analyses can you send a punch biopsy sample for?

A

histology: pattern, shape, cell populations and

inflammatory proteins for antibodies in immunofluorescence

tissue culture

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5
Q

what are the two types of lupus erythematosus

A

systemic lupus erythematosus and cutaneous (discoid) lupus

but they overlap

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6
Q

there are diagnostic criteria for systemic lupus erythematosus. what categories of symptoms do these include?

A

mucocutaneous, involving cartilage and organs, haematological, immunological

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7
Q

what are the mucocutaneous parameters of systemic lupus e.

A

chronic or acute cutaneous lupus, oral ulcers, alopecia

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8
Q

what are the “other organ involvement” parameters of lupus

A

alopecia, synovitis, serositis, renal disorder neuro disorder

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9
Q

spme haematological example parameters for SLE

A

haemolytic anemia thrombocytopenia, leukopenia

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10
Q

immunologival ex. parameters for SLE

A

ana anti ds DNA antiSmit antibodies antiphospholipid low comlement and direct coombs test

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11
Q

skin features of systemic lupus

A

erythematous rash photodistributed(MEANS IN SUN EXPOSED AREAS)

chilblains: itchy swellings ex on toes

livedo reticularis

hairloss
palpable purpura

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12
Q

what do i need to know about the autoantibody profile in dermatomyositis?

A

there are subtypes of dermatomyositis with some different clinical features that can be predicted by aytoantibody profile

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13
Q

other than the antibody tests what other tests can be used to support dermatomyositis diagnosis

A

ANA
CK
skin bipsy
LFT (alt often incr)
EMG (muscle elctrophysiological test)
screen for internal malignancy

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14
Q

what are the broadest classifications of vasculitis

A

small vessel, small and medium vessel, medium and large vessel vasculitis

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15
Q

what are two subtypes of small vessel vasculitis and which usually requires treatment vs the other that doesnt

A

1)cutaneous small vessel (leukocytoclastic) vasculitis: doesn’t usually require treatment
and
2)special types

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16
Q

what are the types of cutaneous small vessel vasculitis

A

idiopathic, from infection, from medication exposure, inflammatory (connective tissue disease)- usually no treatment

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17
Q

examples of special types of small vessel vasculitis

A

IgA vasc. Henoch-Scholelin
urticarial
acute hemorrhagic oedema of infancy
erythema elevatum diutinum

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18
Q

small and medium vasculitis types

A

cryoglobulinemia type 2 and 3

and

ANCA - associated (has subtypes on slide)

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19
Q

medium vasculitis types

A

polyarteritis nodosa (PAN) - BENEIGN CUTANEOUS FORM OR SYSTEMIC FORM

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20
Q

large vessel vasculiutis types

A

temporal arteritis
arteritis tayakasu

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21
Q

what is sarcoidosis and what is an important feature in terms of derm

A

its a systemic (can affect multiple organs) granulomatous (granulomas- collections of immune cells- form in various places in body) disorder

one of its most important features is how variable cutaneous presentations it can have

justfor better understanding from chat gpt: Sarcoidosis is considered to be a multisystem inflammatory disorder, but its exact cause and pathogenesis are not fully understood. While it shares some similarities with autoimmune diseases, such as the involvement of immune cells and inflammation, sarcoidosis is not typically classified as an autoimmune disease.

In autoimmune diseases, the immune system mistakenly attacks the body’s own tissues and cells, leading to inflammation and tissue damage. In sarcoidosis, the immune system mounts an abnormal inflammatory response, characterized by the formation of granulomas—small clumps of immune cells—primarily in the lungs and lymph nodes, but it is not clearly a result of autoimmunity.

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22
Q

what is an organ commonly affected by sarcoidosis

A

lungs

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23
Q

what percentage of sarcoidosis cases have cutaneous presentations

A

33%

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24
Q

what are some examples of the cutaneous manifestations of sarcoidosis

A

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

LUPUS PERNIO (looks like the lupus rash- nothing to do with lupus)

ulcerative

scar sarcoid

erythema nodosum

25
Q

what kind of granulomas are the ones involved in sarcoidosis histologically?

A

histologically non- caseating (no debris and nucleus-less death) epithelioid granulomas

26
Q

58-year old male with a history of multiple myeloma presents with a rash, fever and tachycardia. His renal function is deteriorating and liver function tests deranged.
Was recently treated with co-amoxiclav for suspected infection (source not identified) but there was no improvement so was switched to pipercillin/tazobactam. IF ITS NOt infeciton ehat might it be?

A

drug reaction with eosinophelia and systemic symptoms (DRESS)

27
Q

what is dress in short

A

rash and systemic upset incorporating haematological and solid organ disturbances

28
Q

what are the scoring criterial of DRESS and how does it work

A

all these add up to a score, the higher the more likely it is DRESS

fever over 38.5
lymphadenopathy
circulating atypical lymphocytes
peripheral hypereosinophelia >0.7 x10^9
internal organ involvement
negative ANA, hepatitis/ mycoplasma, chlamydia
skin involvement : >50% BSA, characteristic cutaneous eruption, characteristic biopsy

29
Q

organs that can be involved in DRESS an most common one and most mortality linked one

A

lungs, kidneys, liver: Most common: most death associated with it too, heart, brain, thryroid

30
Q

what are common drugs that cause DRESSand how long after exposure

A

2-6 weeks after exposure,
common drugs: sulfonamides, anti-epileptics, antibiotics, ibuprofen

31
Q

what are the characteristic rashes of DRESS

A

urticated papular exanthem: widespread papules, maculopapular eruption, widespread erythema, head/ neck oedema, erythema multiforme- like

32
Q

treatment of DRESS and mortality

A

withdrawal of drug causing (culprit) -can be hard to identify if started many drugs together

give corticosteroids

5-10%

33
Q

50-year old male with a history of acute myelogenous leukemia. Presents with extensive rash 3 weeks after allogeneic hematopoietic stem-cell transplantation. Also has diarrhoea.
He is on multiple new drugs: pipercillin, tazobactam, mycophenolate mofetil, enoxaparin.
Observation and examination (O&E) reveal extensive rash on face, trunk and limbs – erythematous macules and papules with large areas of confluence ~60% of body surface area (BSA). He also has scleral icterus and oral ulceration.

is this drug induced or immune related and why?

A

its alloimmune: graft vs host disease (GvHD) because of stem cell transplant

we can tell form face involvement, acral involvement, diarrhoea more in this than in drug induced stuff

34
Q

how many people that get stem cell transplants get GvHD

A

10-80%

35
Q

PATHOGENESIS OF GvHD

A

donor-derived T lymphocyte activity against antigens in an immunocompromised RECIPIENT (all stem cell transpl recipients recieve immunosupression)

36
Q

organs mainly affected in GvHD

A

SKIN
LIVER
GI TRACT

37
Q

what is the med term for itch

A

pruritus

38
Q

54-year-old male. Has pruritus (itch) for 1 year but no rash. His symptoms have been worsening and anti-histamines have been unhelpful.
He has no PMH and is not on any medications. Blood tests reveal polycythemia.

is this a skin or internal problem?

A

internal since itch doesnt leave and has no rash

39
Q

what are internal causes of itch

A

haematological: lymphoma, polycythemia

uraemia

cholestasis

iron def or overload

HIV/ HEP A B C

cancer

drugs opiates/ opioids

psychogenic

pruritus of old age

40
Q

what investigations to do for pruritus

A

FBC LDH
renal prof
lft
ferritin
xr chest
HIV/ HEP ABC

41
Q

WHAT happens when you chronically scratch yourself and what is it called

A

dark spots called nodular prurigo

42
Q

what is something to always consider in the onset of flushing?

A

carcinoid syndrome

43
Q

what is carcinoid syndrome

A

signifies metastases of a malignant carcinoid tumour

44
Q

other symptoms of carcinoid syndrome

A

diarrhoea
bronchospasm
hypotension

45
Q

27-year-old male with a history of epilepsy. Presents with flu-like illness, sore eyes and oral ulceration, followed by extensive painful rash. Was recently started on new antiepileptic drug and is on no other medication.

what does this look like

A

Steven Johnsonn Syndrome/ toxic epidermal Necrolysis
SJS/ TEN

46
Q

what is SJS/ TEN adn main symptoms

A

mderm emergency

prodromal flu like symptoms

abrupt onset lesions on trunk more than limbs

macules, blisters, erythema- atypical targetoid that merge and these sheets of skin detach : this is “extendive full thickness mucocutaneous necrosis”- happens 2-3 days after syndrome starts

47
Q

are drugs or infections more common cause of SJS/ TEN

A

drugs

48
Q

difference between names of SJS and TEN

A

SJS is when <10% BSA and TEN is when >30% anything between is SJS/TEN

49
Q

WHAT IS THE underlying pathophysiology in SJS/TEN and how long before rash can it start

A

cell-mediated cytotoxic reaction against epidermal cells - can start up to 3 weeks before rash

50
Q

some drugs that can cause SJS.TEN

A

antibiotics: beta lactams and sulphonamides

allopurinol

antiepileptic drugs: phenzytoin, carbamazepine, lamotrigine

NSAIDS

51
Q

DIFFerentials for SJS/ TEN

A

STAphylococcal scaled skin syndrome SSSS
thermal burns
cutaneous graft vs host disease

52
Q

what is the score used to help asses severity of SJS/ TEN called?

A

SCORTEN

53
Q

WHAT ARE the complications with SJS/TEN

A

death- 30%
blindness, dehydration, hypo/hyperthermia. renal tubular necrosis, eroded GI tract, interstitial pneumonitis, neutropaenia, liver and heart failure

54
Q

what is erythroderma

A

genmeralized erythema affecting >90%BSA,
involving overall impairment in skin function that is reflected in systemic manifestations

55
Q

what are the systemic manifestations of erythroderma?

A

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

56
Q

etiologies of erythroderma

A

drug reactions
cutaneous t cell lymphoma: sezary syndrome
psoriasis
atopic eczema
idiopathic 25-30%

57
Q

management of erythroderma>

A

treat underlying cause,
hospitalise if systemically unwell
restore fluid balance, manage tmp, circulatory status

emollients to support skin barrier

+/- topical steroids
+/-antibiotics

58
Q

is it common to have derm presentations in general systemic diseases?

A

YESS rl important to understand that derm links with systemic diseases, its not just independant

dermatology yr 2 (2 decks)

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