the skin in systemic disease

Question 1

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

Answer 1

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

Question 2

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?

Answer 2

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

Question 3

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

Answer 3

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

Question 4

what analyses can you send a punch biopsy sample for?

Answer 4

histology: pattern, shape, cell populations and

inflammatory proteins for antibodies in immunofluorescence

tissue culture

Question 5

what are the two types of lupus erythematosus

Answer 5

systemic lupus erythematosus and cutaneous (discoid) lupus

but they overlap

Question 6

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

Answer 6

mucocutaneous, involving cartilage and organs, haematological, immunological

Question 7

what are the mucocutaneous parameters of systemic lupus e.

Answer 7

chronic or acute cutaneous lupus, oral ulcers, alopecia

Question 8

what are the “other organ involvement” parameters of lupus

Answer 8

alopecia, synovitis, serositis, renal disorder neuro disorder

Question 9

spme haematological example parameters for SLE

Answer 9

haemolytic anemia thrombocytopenia, leukopenia

Question 10

immunologival ex. parameters for SLE

Answer 10

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

Question 11

skin features of systemic lupus

Answer 11

erythematous rash photodistributed(MEANS IN SUN EXPOSED AREAS)

chilblains: itchy swellings ex on toes

livedo reticularis

hairloss
palpable purpura

Question 12

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

Answer 12

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

Question 13

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

Answer 13

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

Question 14

what are the broadest classifications of vasculitis

Answer 14

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

Question 15

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

Answer 15

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

Question 16

what are the types of cutaneous small vessel vasculitis

Answer 16

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

Question 17

examples of special types of small vessel vasculitis

Answer 17

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

Question 18

small and medium vasculitis types

Answer 18

cryoglobulinemia type 2 and 3

and

ANCA - associated (has subtypes on slide)

Question 19

medium vasculitis types

Answer 19

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

Question 20

large vessel vasculiutis types

Answer 20

temporal arteritis
arteritis tayakasu

Question 21

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

Answer 21

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.

Question 22

what is an organ commonly affected by sarcoidosis

Answer 22

lungs

Question 23

what percentage of sarcoidosis cases have cutaneous presentations

Answer 23

33%

Question 24

what are some examples of the cutaneous manifestations of sarcoidosis

Answer 24

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

Question 25

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

Answer 25

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

Question 26

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?

Answer 26

drug reaction with eosinophelia and systemic symptoms (DRESS)

Question 27

what is dress in short

Answer 27

rash and systemic upset incorporating haematological and solid organ disturbances

Question 28

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

Answer 28

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

Question 29

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

Answer 29

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

Question 30

what are common drugs that cause DRESSand how long after exposure

Answer 30

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

Question 31

what are the characteristic rashes of DRESS

Answer 31

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

Question 32

treatment of DRESS and mortality

Answer 32

withdrawal of drug causing (culprit) -can be hard to identify if started many drugs together give corticosteroids 5-10%

Question 33

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?

Answer 33

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

Question 34

how many people that get stem cell transplants get GvHD

Answer 34

10-80%

Question 35

PATHOGENESIS OF GvHD

Answer 35

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

Question 36

organs mainly affected in GvHD

Answer 36

SKIN LIVER GI TRACT

Question 37

what is the med term for itch

Answer 37

pruritus

Question 38

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?

Answer 38

internal since itch doesnt leave and has no rash

Question 39

what are internal causes of itch

Answer 39

haematological: lymphoma, polycythemia uraemia cholestasis iron def or overload HIV/ HEP A B C cancer drugs opiates/ opioids psychogenic pruritus of old age

Question 40

what investigations to do for pruritus

Answer 40

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

Question 41

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

Answer 41

dark spots called nodular prurigo

Question 42

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

Answer 42

carcinoid syndrome

Question 43

what is carcinoid syndrome

Answer 43

signifies metastases of a malignant carcinoid tumour

Question 44

other symptoms of carcinoid syndrome

Answer 44

diarrhoea bronchospasm hypotension

Question 45

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

Answer 45

Steven Johnsonn Syndrome/ toxic epidermal Necrolysis SJS/ TEN

Question 46

what is SJS/ TEN adn main symptoms

Answer 46

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

Question 47

are drugs or infections more common cause of SJS/ TEN

Answer 47

drugs

Question 48

difference between names of SJS and TEN

Answer 48

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

Question 49

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

Answer 49

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

Question 50

some drugs that can cause SJS.TEN

Answer 50

antibiotics: beta lactams and sulphonamides allopurinol antiepileptic drugs: phenzytoin, carbamazepine, lamotrigine NSAIDS

Question 51

DIFFerentials for SJS/ TEN

Answer 51

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

Question 52

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

Answer 52

SCORTEN

Question 53

WHAT ARE the complications with SJS/TEN

Answer 53

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

Question 54

what is erythroderma

Answer 54

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

Question 55

what are the systemic manifestations of erythroderma?

Answer 55

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

Question 56

etiologies of erythroderma

Answer 56

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

Question 57

management of erythroderma>

Answer 57

treat underlying cause, hospitalise if systemically unwell restore fluid balance, manage tmp, circulatory status emollients to support skin barrier +/- topical steroids +/-antibiotics

Question 58

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

Answer 58

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