(derm) managing skin disorders in systemic disease

Question 1

look at the following images

which condition below is not associated with the pathology indicated?

a) diabetes mellitus
b) digital ischaemia
c) malignancy
d) interstitial lung disease

Answer 1

diabetes mellitus is not associated with the signs of dermatomyositis shown

digital ischamia = subtype with anti-MDA5 antibodies

malignancy = anti-p155 antibodies

interstitial lung disease = subtype with anti-MDA5 antibodies

Question 2

can dermatomyositis be associated with diabetes mellitus?

Answer 2

if the dermatomyositis patients are treated with prednisolone = can lead to the development of diabetes mellitus

Question 3

what are Gottron’s papules?

Answer 3

red or violet bumps that form on the outside joints of the hand specifically at the points of the metacarpophalangeal and the interphalangeal joints

Question 4

what is Shawl sign?

Answer 4

characteristic erythematous rash distributed in a ‘shawl’ pattern over the neck, upper back, chest and shoulder

Question 5

which of the following are associated with chronic kidney disease?

a) corkscrew hairs and perifollicular purpura
b) calciphylaxis
c) Casal’s necklace
d) periocular purpura

Answer 5

b) calciphylaxis

corkscrew hairs and perifollicular purpura = associated w scurvy

calciphylaxis = associated w CKD

Casal’s necklace = associated w vitamin B3 (niacin) deficiency

periocular purpura = associated w dermatomyositis/systemic amyloidosis

Question 6

explain how chronic kidney disease leads to calciphylaxis

Answer 6

altered calcium metabolism

= deposition in arterioles (calcium accumulates in small blood vessels of the fat and skin tissues)

= necrosis in the skin it supplies

= calciphylaxis

Question 7

what are corkscrew hairs and perifollicular purpura commonly associated with?

Answer 7

scurvy

Question 8

what is Casal’s necklace commonly associated with?

Answer 8

pellagra (vitamin B3 deficiency)

Question 9

what is periocular purpura commonly associated with?

Answer 9

systemic amyloidosis

Question 10

which of the following statements about the disease shown are incorrect?

a) complications include blindness
b) pain is a characteristic feature and diagnostic clue
c) lymphadenopathy is a characteristic clue
d) extension into the GI tract may occur

Answer 10

c) lymphadenopathy is a characteristic clue

the condition shown is SJS/TEN and lymphadenopathy is not characteristic of this but is part of the diagnostic criteria for DRESS

Question 11

what must you ensure when treating someone with SJS/TEN?

Answer 11

involve opthalmology in every case even if they don’t have ocular involvement at presentation to prevent the devastating complication of blindness

+ must assess for pain

Question 12

what does the following image show?

Answer 12

denudation = loss of surface layers, such as the epithelium

(epidermal necrosis = hallmark of SJS/TEN)

Question 13

which of the following is true with regards to an itch in the absence of a rash?

a) indicates malingering or a psychogenic aetiology
b) chronic rubbing or scratching may cause the appearance of nodules
c) drugs are an unlikely culprit
d) iron deficiency, not iron overload causes an itch

Answer 13

b) chronic rubbing or scratching may cause the appearance of nodules = NODULAR PRURIGO

a = can have multiple causes, not just one

c = drugs, including opiates, frequently cause pruritus in the absence of a rash

d = both can cause itching

Question 14

which of the following is correct regarding Drug Reaction with Eosinophilia and Systemic Symptoms?

a) the brain is not among the organs targeted
b) the complications of erythroderma account for most of the fatalities
c) characteristic features on biopsy allow diagnostic confirmation
d) fulminant liver failure may occur

Answer 14

d) fulminant liver failure may occur

a = brain is targeted in DRESS

b = fulminant liver failure accounts for most fatalities

c = diagnosis is based on symptoms in a criteria AND biopsy (contributes to, not determines, the diagnosis)

Question 15

which of the following is a manifestation of sarcoidosis?

a) lupus vulgaris
b) lupus profundus
c) lupus pernio
d) lupus tumidus

Answer 15

lupus pernio = bluish-red or violaceous nodules and plaques over the nose, cheeks and ears

a = form of cutaneous tuberculosis

b = form of cutaneous lupus erythematosus

d = variant of cutaneous lupus erythematosus

Question 16

what is lupus vulgaris?

Answer 16

form of cutaneous tuberculosis

Question 17

what is lupus profundus?

Answer 17

form of cutaneous lupus erythematosus affecting subcutaneous fat

Question 18

what is lupus tumidus?

Answer 18

a variant of cutaneous lupus erythematosus

Question 19

which of the following nail changes are not associated with chronic liver disease?

Answer 19

half and half nails are NOT associated with chronic liver disease, but instead with chronic kidney disease

= may be associated w Crohn’s or a normal variant

(clubbing = wide differential diagnoses; muehrcke’s lines = transverse white lines on the nails; terry’s nails = whitened nail bed with a rim of erythema at the distal end)

Question 20

which of the following conditions is not associated with inflammatory bowel disease?

a) pyoderma gangrenosum
b) dermatitis herpetiformis
c) hidradenitis suppuritiva
d) orofacial granulomatosis

Answer 20

dermatitis herpetiformis (sssociated with coeliac disease)

(all others associated w IBD)

Question 21

which of the following are not a manifestation of malignancy?

a) lues maligna
b) pyoderma gangrenosum
c) acanthosis nigricans
d) dermatomyositis

Answer 21

a) lues maligna = necrotic form of secondary syphilis

pyoderma gangrenosum = has multiple associations, including malignancy

acanthosis nigricans = may be associated with internal malignancy or insulin resistance

dermatomyositis = can be paraneoplastic

Question 22

in which of the following forms does HIV seroconversion manifest?

a) urticaria
b) erythema multiforme
c) morbilliform rash
d) all of the above

Answer 22

all of the above

HIV seroconversion has a many variable and non-specific manifestations, necessitating a low threshold for testing

(e.g. another one is genital or oral ulceration)

Question 23

which of the following conditions may be indicative of underlying acromegaly?

a) cutis gyrata verticis
b) xerosis
c) perforating disorder
d) erythema gyratum repens

Answer 23

a) cutis gyrata verticis

xerosis = dryness occurs in many conditions, such as: diabetes, renal failure, but NOT acromegaly

perforating disorder = classically observed in diabetes, renal failure

eythema gyratum repens = a paraneoplastic type of annular erythema with a wood-grain appearance

Question 24

what is xerosis and when does it occur?

Answer 24

xerosis = dryness occurs in many conditions, such as diabetes, renal failure, but NOT acromegaly

Question 25

what is perforating disorder and when is it observed?

Answer 25

perforating disorder = classically observed in diabetes, renal failure

Question 26

what is erythema gyratum repens?

Answer 26

eythema gyratum repens = a paraneoplastic type of annular erythema with a wood-grain appearance

Question 27

which of the following statements are true regarding the skin in systemic disease? ## Footnote a) comprehensive diagnostic investigations must be performed in each rash to avoid missing underlying systemic conditions, as history is not systemic or specific b) spot diagnoses indicate good clinical skills c) dermatological diagnoses are desirable, but not essential so taking a skin biopsy instead allows the histopathologic to make the diagnosis d) management of systemic diseases can be undertaken by several specialties concurrently

Answer 27

d) management of systemic diseases can be undertaken by several specialties concurrently = management should be taken by all relevant specialties in a multidisciplinary manner a = investigation should be guided by clinical skills to prevent false positives/negatives b = diagnosis requires comprehensive clinical assessment +/- diagnostic tests and considerations of differentials c = histological features are often non-specific/non-diagnostic

Question 28

which of the following renders GVHD (graft vs host disease) a more likely cause of this rash than drug consumption? a) acral involvement b) facial involvement c) diarrhoea d) all of the above

Answer 28

d) all of the above in a clinically equivocal rash in a allogenic HSCT (haemopoietic stem cell transplant) recipient, diarrhoea + facial + acral involvement all suggest cutaneous GVHD is a more likely cause than drugs

Question 29

which of the following investigations is indicated for this presentation? (new onset) fever, rash, arthritis, no new medication A) urinalysis B) vitamin C C) lipid panel D) HbA1c

Answer 29

A) urinalysis = suspect systemic lupus erythematosus (also do FBC, U&E, ANA, anti-dsDNA, complement tests)

Question 30

which of the following investigations is indicated in this newborn patient with a rash? A) genetic testing B) syphilis C) ECG D) skin biopsy

Answer 30

C) ECG = annular rash characteristic of neonatal lupus = 50% risk of complete heart block and consequent heart failure (associated with Ro and La antibodies)

Question 31

what does this neonatal annular rash indicate and why is this dangerous?

Answer 31

= annular rash characteristic of neonatal lupus = 50% risk of complete heart block and consequent heart failure

Question 32

which antibodies are associated with this neonatal rash?

Answer 32

associated with Ro and La antibodies

Question 33

which blood test would be done for a patient that presents with the following condition?

Answer 33

acanthosis nigricans do a HbA1c test to assess blood glucose levels for prediabetes or diabetes

Question 34

which blood test would be done for the following presentation?

Answer 34

corkscrew hairs = indicative of vitamin C deficiency/scurvy so do a vitamin C test

Question 35

which blood test would be done for the following presentation?

Answer 35

lipid profile = presentation of eruptive xanthelasma (white/yellow lumps of fatty material) is indicative of hyperlipidaemia

Question 36

for which condition is this a common presentation?

Answer 36

epidermal necrosis (denudation) is a common presentation for SJS/TEN

Question 37

what is shown in the following picture and how does this develop?

Answer 37

nodular prurigo = can develop due to chronic rubbing or scratching

Question 38

what do the following nails indicate?

Answer 38

half and half nails = usually indicate chronic kidney disease or Crohn's disease but can appear in someone who is completely normal and healthy

Question 39

what do the following nails suggest?

Answer 39

clubbing = has a very wide differential diagnosis (e.g. hypoxia, liver disease, cardiovascular disease, lung disease etc)

Question 40

what does the following presentation suggest?

Answer 40

cutis gyrata verticis = indicative of underlying acromegaly

Question 41

what is the following presentation classically a sign of?

Answer 41

perforating disorder = classically seen in diabetic renal failure