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Flashcards in Skin and Systemic disease Dan Deck (127):
1

T/F
Hepatitis B infection can be associated with urticaria, Gianotti-Crosti syndrome and polyarteritis nodosa

T

2

40% of type II cryoglobulinaemia is HCV associated

F
70-90%

3

Hepatitis B is associated w Necrolytic acral erythema

F
Hep C

4

Necrolytic acral erythema appears as well-circumscribed, dusky erythematous plaques with adherent scale

T

5

The connection between lichen planus and hepatitis C infection may actually be due to geographical variation

T

6

What are the cutaneous features of chronic liver disease?

Acral erythema
Spider naevi + telangiectasia
Dilated abdominal/chest veins - caput medusa
Nail – clubbing, pallor, Muehrcke’s lines
Bruising, purpura
Jaundice
Thin skin, striae
Excoriations
Features of malnutrition
Associated lesions
- xanthomas
- PCT
- Vasculitis
- LP

7

Pigmentation in haemochromatosis is due to haemosiderin deposition in the skin

F
melanin

8

Strongest risk factors for expression of PCT are HCV infection and homozygosity for C282Y (haemochromatosis) mutation

T

9

Jaundice is first seen as a yellowish hue of the sclerae and soft palate

T

10

Carotenaemia also discolours the sclerae

F

11

Acquired zinc deficiency in adults may be characterised by an reticulate eczema, erosions in the perianal and genital areas, cheilitis, hair loss and Beau’s lines

T

12

Acrodermatitis enteropathica is AD

F
AR

13

Cullens and Grey-Turners signs are the most common skin changes associated with pancreatitis

F
Jaundice and panniculitis are

14

Pancreatic panniculitis can be caused by acute or chronic pancreatitis, pancreatic carcinoma, pancreatic pseudocyst, traumatic pancreatitis

T
any cause of pancreatitis or pancreatic insult.
1/3 are caused by pancreatic carcinoma, so must always be aware

15

Trousseau’s sign describes a Sister Mary Joseph nodule when it occurs in conjunction with pancreatic carcinoma

F
10% of cases of sister mary joseph nodule is a metastasis due to pancreatic carcinoma
Trousseau’s sign describes multiple, superficial migratory thrombophlebitis and is classically associated with Lung cancer (most often) and pancreatic ca (25%)

16

Necrolytic migratory erythema occurs in association w malignant adrenal carcinoma

F
hyperglucagonaemia – glucagonoma – alpha-cell tumour in tail of pancreas
Other causes include
- pancreatic insufficiency
- intestinal malaborsoprtion or protein loss
- liver cirrhosis
- aberrant glucoagon secreting tumours – bronchial or nasopharyngeal

17

Glucagonomas occur in MEN syndrome

T
Classically MEN type I but also MEN type II

18

Skin changes persist following removal of glucogonoma

F

19

Clinical features of Necrolytic migratory erythema include an itchy, burning rash on flexural sites including lower abdomen, groin, buttocks and thighs

T

20

Wht are systemic features of glucagonoma?

Weight loss
Anaemia
Diabetes
Diarrhoea
Weakness
Venous thrombosis
Psychiatric disturbances
Zinc deficiency

21

Marked spongiosis and hyperkeratosis are typically seen in necrolytic migratory erythema

F
mild spongiosis and dyskeratosis
superficial perivascular inflammation
Clefts and necrotic keratinocytes and cellular debris

22

Clinical features of Necrolytic migratory erythema include an itchy, burning rash on flexural sites including lower abdomen, groin, buttocks and thighs

T
fragile bullae and vesicles, irregular centrifugal annular lesions which can crust
has fluctuating course of cyclical pattern
angular cheilitis and glossitis

23

Glucagonoma can be treated with somatostatin analogues

Octreotide – improves rash and symptoms by altering glucagon metabolism
Lantreotide can last for 2 weeks

24

Fabry’s disease is due to alpha-galactosidase A deficiency

T

25

Fabry’s disease can be treated using synthetic alpha-galactosidase

T

26

Fabrys get renal disease and other systemic manifestations due to deposition of neutral glycosphingolipids in vascular endothelium and tissue

T

27

Exostoses arising from the posterior aspect of the iliac bones ("iliac horns") are considered pathognomonic of Nail Patella syndrome

T
occur in about 80%
considered pathognomonic

28

Which genodermatoses associated w renal carcinoma?

Birt-Hogge-Dube syndrome
Hereditary leiomyomatosis and renal cell carcinoma (HLRCC, Reed syndrome)
Cowden’s disease
von Hippel-Lindau syndrome
Muir-Torre (renal pelvis transitional epithelium - all others are RCC)

29

Cutaneous features of systemic amyloidosis include waxy, purpuric lesions as well as hepatomegaly, oedema and carpal tunnel

T

30

All patients who present with cutaneous vasculitis should have a urine dipstick and serum creatinine and eGFR checked

T

31

Kyrle’s disease is a reactive perforating collagenosis associated w renal failure

T
also associated w diabetes
Very itchy

32

Pruritus in renal failure is often associated w hyperuricaemia

T

33

Nephrogenic systemic fibrosis is associated w gadolinium containing radiocontrast dyes used in patients with chronic renal failure

T

34

Patients with liver transplants appear to have a higher incidence of cutaneous malignancy than renal transplant patients

F
– vice versa. Due to higher immunosuppression required + inc longevity post transplant

35

Post-renal transplant, patients BCC risk increases up to 250 fold

F
BCC risk by inc 10x
SCC risk by inc 65–250x

36

Uncontrolled expression of HPV infection may be a co-factor in the increased risk of SCC in immunosuppressed transplant patients

T

37

90% of children with Kawasaki disease develop coronary artery aneurysms

F
25%

38

The cardiac muscle is classically the only organ not affected in systemic sclerosis

F
involved in 80%

39

What are the Stigmata of subacute bacterial endocarditis?

Septic emboli or immune complex cause skin findings
purpuric, pustular or erythematous
non-specific small vessel vasculitis
spinter haemorrhages
Roth sponts – haemorrhages in conjunctivae and retina
Oselrs nodes – small, tender, red papules on distal phalanx
Janeway lesions – faint, red, macular lesions on thenar and hypothenar eminences

40

What are Features of Carney complex?

Skin – naevi + blue naevi, lentigines, ephelides, myxoid neurofibromas
Pigmented adrenocortical nodules – adrenal hyperplasia and is therefore a rare cause of ACTH independent Cushing’s syndrome
Precocious puberty in females
Atrial myxomas
Also
- GH-secreting pituitary tumours
- Sertoli cell and leydig cell testicular tumours
- Thyroid cysts
- Ovarian cysts and tumours

41

Systemic sclerosis may be associated w fibrosing alveolitis and interstitial fibrosis

T

42

Loeffler’s syndrome describes pulmonary infiltrates and peripheral eosinophilia and is often due to parasitic infection

T

43

Relapsing polychondritis is due to autoantibodies against type III collagen

F
type II

44

Dermatomyositis is usually related to anti-La antibodies

F
anti-Jo1

45

Yellow nail syndrome occurs without associated systemic features

F
pleural effusion, bronchiectasis, chronic lung infections, empyema, lymphoedema, sinus infections

46

Sickle cell anaemia and thalassaemia can be causes of recalcitrant lower leg ulcers

T

47

Aquagenic pruritus appears in polycythaemia rubra vera with RBC counts above 200g/L

F
aquagenic pruritus can predate the diagnosis of PCV
- follow up any aquagenic pruritus pts and reinvestigate regularly

48

Erythromelalgia is associated with sickle cell anaemia

F
with PCV

49

Eosinophilia myalgia syndrome is an idiopathic fibrotic condition

F
associated with ingestion of tryptophan

50

Wells’ syndrome may clinically mimic bacterial cellulitis, but has a dense eosinophilic infiltrate

T

51

Hypereosinophilic syndrome has eosinophilis in excess of 1500/uL w no apparent cause for greater than 6/12

T
Can have endomyocardial fibrosis, eosinophilic vasculitis, thrombosis, cutaneous lesions

52

Late onset CAH is the most common AR disorder in humans

T

53

which skin diseases are associated w leukaemia?

Sweet’s syndrome
Neutrophilic dermatosis of dorsal hands
Pyoderma gangrenosum
Subcorneal pustular dermatosis
EED
Neutrophilic eccrine hidradenitis
Vasculitis
Eosinophilic pustular folliculitis erythroderma
JXG w NF-1 - juvenile chronic myelomonocytic leukaemia
Leukaemia cutis
Relapsing polychondritis - MDS

54

Leukaemia cutis is more common in chronic leukaemia than acute

F
more common in acute [AML]
Although can occur in any chronic and may herald a blast transformation

55

Coumarin necrosis is due to a temporary reduction in levels of vitamin K dependent natural anticoagulants causing a temporary state of thrombophilia

T

56

Warfarin necrosis is more common in Males

F
females

57

Which Genodermatoses are associated w haematological malignancy?

Chediak higashi
Wiskott Aldrich
NF-1 + JXGs
Dyskeratosis congenita
Fanconi’s anaemia
Ataxia telangiectasia
Werner's, Bloom's and Rothmund-Thomson

58

Erythematous plaque or urticated lesion with expands with central clearing occur in erythema chronicum migrans

T

59

After the initial lesion, crops of vesicles can develop at sites distant to erythema chronicum migrans site

F – lesions resembling EAC or EM can develop. Smaller than initial lesion

60

Histologically erythema chronicum migrans shows superficial and deep perivascular lymphocytic infiltrate around vessels and appendages w occasionally plasma cells

T

61

erythema marginatum rehumaticum clinically consists of rings or segments of rings, pale or dull red in colour which can fade in a few hours and occur in recurrent crops in different sites

T

62

Erythema gyratum repens is always associated with malignancy

F
80% of the time
Has also been associated with CREST syndrome, tuberculosis, drug hypersensitivity

63

Erythema gyratum repens is most commonly associated w lung cancer

T – 33%
then bowel, oesophagus, urogenital, breast, pancreas, haematological

64

Erythema gyratum repens is thought to be immunologically mediated

T – supported by deposition of C3 or IgG in sublamina densa

65

Erythema gyratum repens clinically resembles the grain of wood

T

66

DDx for Erythema gyratum repens

TEN LL
T = Tinea imbricata
E = Erythrokeratoderma variabilis
N = Necrolytic migratory erythema
Lupus
Lapiere's Subacute annular variant of psoriasis

67

Annular erythema of infancy is really just EAC in children

T

68

In annular erythema of infancy and infective source can be sought and treated, often with a good response of cutaneous lesions

T

69

Annular erythema associated with extractable nuclear antigens is seen in Lupus, Sjogren’s disease, Neonatal lupus

T
and is associated with anti-SSA and anti-SSB antibodies

70

Common associations with erythema annulare centrifugum are fungal infections, other viral infections, drugs, lymphoma

T

71

Histologically EAC shows perivascular ‘sleeve-like’ lymphohistiocytic infiltrate

T
'coat sleeving'

72

Erythema annulare centrifigum appears as a small, pink, infiltrated papule, which gradually enlarges as a ring

T
and spreads leaving a trailing scale

73

What is the treatment of Treatment for EAC?

Discovery and elimination of cause if possible
Antihistamines
Topical corticosteroids
Vitamin D
Narrow-band UVB
? systemic immunosuppressants

74

Facial features of acromegaly include prognathism, frontal bossing, oily skin, large and furrowed tongue, triangular ears

T
Also skin shows;
Acanthosis nigricans
Addisonian like pigmentation due to increased MSH
Cutis verticis gyrata (secondary)

75

Pituitary insufficiency may manifest as hyperpigmentation due to uncontrolled secretion of MSH

F
Decreased MSH – hypopigmentation, but mucous membranes retain normal colour
Also inc sunburning and lack of tanning

76

Pituitary Cushing’s disease causes hyperpigmentation as secretion of pro-opiomelanocortin causes overproduction of ACTH and MSH

T

77

What are screening tests for Cushing’s disease

8 am cortisol
24-hour urinary cortisol repeated 2x or
1mg overnight dexamethasone suppression tests
- values 4 x normal

78

Nelson’s syndrome is eponymous for empty sella syndrome

F
Nelson’s syndrome is the rapid growth of pituitary adenomas following adrenalectomy

79

Secondary adrenal insufficiency due to hypothalamic or pituitary insufficiency lacks pigmentary changes

T

80

Describe features of Addisonian pigmentation

Slow onset
Light exposed areas – face, dorsa of hands
Accentuation in genital, perineum, axillae, areolae, any areas subject to friction (eg elbows, knees, under clothing)
Scars
Tongue, mucous membranes
Darker hair and longitudinal melanonychia
Eruptive lentigines and darkening existing pigmented lesions

81

Addisonian pigmentation can be seen in primary adrenal insufficiency, Nelson’s syndrome, tumour casuing ectopic ACTH secretion, Cushings disease

T

82

In congenital adrenal hyperplasia and late onset adrenal hyperplasia 17-hydroxyprogesterone, cannot be converted to pogesterone

F
cannot be converted 11-deoxycortisol

83

Congenital adrenal hyperplasia is associated with endogenous steroid induced acne

F
acne in CAH is due to excessive androgens
Other symptoms include
Premature pubic hair
Hirsuitism
Acne
Temporal baldness
Infertility
Accelerated growth spurt and diminished final height in males and females

84

What are Nail changes seen in hyperthyroidism?

Thin, onycholysis (Plummer’s nails), fast growing
Plummer’s = onycholysis + koilonychias; begins on 4th finger

85

Diamond’s triad which is associated w Graves disease refers to what?

pretibial myxoedema, thyroid acropachy, exophthalmos

86

Pretibial myxoedema develops in patients with hypothyroidism

F
hyperthyroidism

87

Histologically pretibial myxoedema may be seen as a thickened dermis with increased collagen

F
thickened dermis, w extensive deposits of acid mucoplysaccharides acausing separation of collagen fibres

88

Thyroid acropachy refers to the eye changes seen in Graves disease

F
clubbing of fingers and toes in associated w soft tissue swelling of hands and feet w/ periosteal bone formation

89

Cutaneous findings of hypothyroidism include pale, cold scaly skin w asteatotic eczema and anhidrosis

T

90

Loss of the medial third of the eyebrow – Hertog’s sign, is common in hypothyroid patients

F
loss of lateral third

91

The main dermatological clinical signs seen in parathyroid disease are related to calciphylaxis and ossification

T
or angiofibromas if MEN1

92

Paget’s disease of the breast is thought to be due to direct epidermal extension of an underlying ductal squamous cell carcinoma

F
adenocarcinoma

93

25% of EMPD have underlying cancer

T

94

Extramammary paget’s disease can be from direct extension from carcinoma of underlying apocrine glands or from a distant tumour

T

95

Most common source of cutaneous metastases are upper aerodigestive tract

F
melanoma and breast ca

96

Sister Mary Joseph’s nodule is most commonly related to bowel tumours

T

97

Cutaneous metastases usually have a poor prognosis and indicate disseminated disease

T

98

What neoplasias are associated w Gorlins syndrome?

BCCs
Rhabdomyosarcoma
Medulloblastoma (in 3% of Gorlins. Also 3% of medulloblastoma pts have Gorlin’s)
Astrocytoma
Meningioma
Fibrosarcomas
Uterine fibroids, desmoids and sarcomas
Ovarian fibroma
Cardiac fibroma

99

CDKN2A mutation is associated with melanoma and renal cell carcinoma

F
Not RCC.
Melanoma
Breast
Thyroid
GIT
SCCs
Larynx
Pancreas
Ocular

100

Cowden’s syndrome is not associated w increased risk of cutaneous malignancy

F
Inc melanoma risk
also thyroid, breast, uterine, GU

101

Muir Torre syndrome is associated with increased risk of thyroid carcinoma

F

102

Uterine cancer is the cancer most strongly associated with Acanthosis nigricans

F
gastric adenocarcinoma

103

Tripe palms alone (esp with clubbing) are indicative of bronchial carcinoma

T

104

Sign of Lesar-Trelat (eruptive Seborrheic keratosis) is highly indicative of internal malignancy

F
only weak association
May also occur in HIV infection, acromegaly and in resolving phase of erythrodermic dermatoses
- MEAL
M = Malig
E = Erythroderma
A = AIDs
L = Lymphoproliferative 20%

105

Acrokeratosis Neoplastica effects the hands and feet only

F
all peripheries inc tip of nose helices of ears, hands and feet and may then become generalised

106

Bazex syndrome (AN) can occur without underlying malignancy

F
this is a required criterion for diagnosis
- most common in SCC or upper respiratory tract or GIT and w metastases to cervical lymph nodes

107

Multicentric reticulohistiocytosis is associated with solid tumour

T
25% of cases
Papules around nail folds – coral bead sign, symmetrical polyarthritis

108

Which haematological disorder is erythromelalgia linked with?

Polycythaemia rubra vera
Essential thrombocytaemia

109

Actinic cheilitis is a cutaneous manifestation of Crohn’s disease

F
Granulomatous cheilitis is

110

What are skin features associated with IBD?

HS
EBA (rare)
Erythema nodosum
Aphthous ulceration
Erythema multiforme
Urticaria and angio-oedema
Neutrophilic dermatosis – pyoderma gangrenous and sweets + Pyodermatitis-pyostomatitis vegetans
Vasculitis and intravascular coagulation
Sarcoid-type lesions at remote sites
Granulomatous cheilitis
Non specific skin changes secondary to malabsorption
Anal and perianal conditions
o Maceration
o Erosion
o Pruritis ani
o Sin tags
o Secondary infection
o ‘metastatic’ granulomatous plaques
o Perianal abscess
o Fissure in ano
o Fistula in ano
Spreading ulceration of perineum and buttocks post colectomy
Skin changes around stoma sites (PG or cur Crohns)
Genital lesions
o Men
- Balanitis
- Posthitis
- Chronic penile lymphoedema and granulomatous lymphangiitis
- Women
- Vulval lesions

111

What are Bywater’s lesions?

Feature of RA
Nailfold thrombosis and infarction causing purpuric macules/papules on distal digits (esp. digital pulp) they are painless

112

Pyoderma gangrenosum is more common in patients with ulcerative colitis than those with Crohn’s disease

T
5% of all UC pts and parallels the GI disease
erythema nodosum is alos more often seen in UC than Crohns

113

Whats the pancreatic panniculitis and polyarthritis syndrome?

Pancreatitis
Arthritis (60%)
Nodular perforating panniculitis
Cancer of pancreas – more common cause than pancreatitis
Radiographic changes – osteolytic bone lesions from marrow necrosis (10%)
Eosinophilia – Schmidt’s triad is arthritis, nodules and eosinophilia
Amylase, lipase and triptase elevated
Serositis – pleuropericarditis often w/ fever

114

What is Jellinek's sign?

hyperpigmentation of eyelid skin in hyperthyroidism

115

What is Pemberton's sign?

red face on raising arms due to a large retrosternal goitre compressing the vena cava and obstructing venous return to the heart when the arms are raised

116

What is Maroni’s sign?

erythema of skin over the thyroid in hyperthyroidism

117

What is EMO syndrome?

Exophthalmos, pretibial Myxoedema, Osteoarthropathy; a rare complication of Graves disease

118

exopthalmos is rarely seen in pts with pretibial myxoedema

F
almost always seen
if these are present look for acropachy (Diamonds) and for osteoarthropathy (EMO)

119

Diabetic cheiroarthropathy does not respond to improved glycaemic control

F
gets better after period of tight control

120

Plummer-Vinson Syndrome is atriad of;
Dysphagia + iron deficiency + esophageal webs

T
middle aged women; rare

121

What are the mucocutaneous findings of Plummer-Vinson Syndrome?

Brittle nail koilonychias
Angular chelitis
Pale atrophic buccal mucosa
Leukoplakia
Glossitis

122

Hermansky Pudlak, Griscelli and Chediak Higashi syndromes are all at risk of haem malignancy

F
Only Chediak Higashi is
Other 2 are at risk of;
hemophagocytic lymphohistiocytosis - a type of cytokine storm involving lymphocytes and macrophages

123

Which derm conditions are associated with bowel polyps?

Peutz-Jeghers syndrome
Gorlin's
Gardner’s syndrome
Cowden’s disease (multiple hamartoma and neoplasia syndrome)
Bannayan-Riley-Ruvalcaba syndrome
Cronkhite-Canada Syndrome (non-inherited)
Birt-Hogg-Dube syndrome
Neurofibromatosis

124

Which derm conditions are associated with bronchiolitis obliterans?

Paraneoplastic pemphigus
chronic GVHD
Sweets

125

Which genoderms get lipomas?

Cowdens
Bunyan –Riley- Ruvalcaba syndrome
Neurofibromatosis
Gardner’s syndrome
Proteus syndrome
MEN 1
+ specific lipomatosis syndromes

126

What dermatoses are associated with Hep B or C?

Small vessel vasculitis (B, C)
Serum sickness-like reaction (B, C)
Urticaria (B, C)
Porphyria cutanea tarda (B, C)
Pruritus (B, C)
Erythema multiforme (B, C)
Erythema nodosum (B>C)
Polyarteritis nodosa (B [classic]>C)
Gianotti–Crosti syndrome (B>C)
Sarcoidosis (with interferon and/or ribavirin therapy; C>B)
Cryoglobulinemic vasculitis (C>B)
Necrolytic acral erythema (C)
Lichen planus – particularly erosive oral disease (C)
Livedo reticularis (C)

127

What are the derm associations of coeliac disease?

DH
Psoriasis
IgA pemphigus
Alopecia
Mouth ulcers
Thrombocytopenic purpura
Systemic associations with possible skin manifestations;
SLE
RA
Sjogrens
Sarcoidosis
Diabetes
AI thyroid disease
Addisons
Anaemia
Autoimmune hepatitis, PBC, PSC
MS and neuropathies