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Derm 2020 > Metabolic & Systemic Disorders > Flashcards

Metabolic & Systemic Disorders Flashcards

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Dermatology Board Prep flashcards
1
Q

Types of xanthomas

A

TV TED (PXP)
Tendinous
Verrucous

Tuberous
Eruptive
Dyslipidaemic (planar, palmar, xanthelasma)

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2
Q

Eruptive xanthoma occurs in setting of secondary not primary dyslipidaemia

A

False , both

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3
Q

Exacerbating factors for eruptive xanthomas

A 
Obesity
High caloric intake
Alcohol abuse
DRUGS - oral oestrogen replacement
Systemic meds: retinoids, protease inhibitors, olanzapine
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4
Q

Plane xanthomas are associated with monoclonal gammopathy

A

True

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5
Q

Verruciform xanthoma occurs in the setting of?

A 
Lymphoedema
EB, pemphigus
DLE
GVHD
Cutaneous lesions lif X linked CHILD syndrome
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6
Q

Management of xanthomas

A

Dietician input
Medications include statins, fibrates, ezetimibe, nicotine acid lowers cholesterol , LDL cholesterol and triglycerides and incr HDL cholesterol
Cholestyramine NOT as effective

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7
Q

Amyloidosis -primary systemic -cutaneous features

A 
Pinch purpura
Macroglossia (carpal tunnel syndrome is association with this is a classic presentation)
Periorbital purpura 
Waxy papulonodules and plaques
Ecchymoses
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8
Q

AMYLOID deposits in macular, lichen amyloidosis and nodular amyloidosis are all keratinocyte derived

A

False , in nodular amyloidosis, they are derived from immunoglobulin light chains and accompanied by an infiltrate of plasma cells

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9
Q

What are some genetic forms of amyloidosis

A 
Familial amyloid polyneuropathy
Muckle wells, 
CAPS
FMF
TRAPS
Familial amyloidosis Finnish type (AD)
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10
Q

Lichen amyloidosis Often occurs on lower legs of south East Asian individuals

A

True

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11
Q

What are the variants of lichen amyloidosis

A

Anosacral
Bullous
Dyschromic (guttate leukoderma superimposed on BG hyperpigmentation)

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12
Q

DDx waxy papules

A 
Papular mucinosis, scleromyxoedema
Nodular mucinosis
Lipoid proteinosis
Adnexal tumours of the face
Colloid milia
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13
Q

Primary systemic amyloidosis patients fulfil criteria for multiple myeloma

A

False - they have an underlying plasma cell dyscrasia

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14
Q

Types of calcinosis cutis

A

Dystrophic - secondary to local tissue damage or altered elastin, collagen. Includes tumours eg. Pilomatricoma , DTE, inclusion cyst
Iatrogenic - extravasated calcium or phosphate infusions
Metabolic - abnormal calcium and/or phosphate metabolism causing precipitation of salts eg. Sarcoidosis, hyperparathyroidism , cutaneous LE, DM, CREST
Idiopathic - without trauma or metabolic defect

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15
Q

Calcinosis cutis develops in <5% of adult pt with DM

A

False 10-20%

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16
Q

Risk factors for calciphylaxis

A 
Female
Obesity
Dialysis
End stage renal disease
Warfarin
DM
Antiphospholipid antibodies
Thrombophlebitis eg. Protein C or S deficiency
Alterations of Vit D dependent anti calcification proteins
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17
Q

5 genetic disorders with dystrophic calcification

A 
Ehler Danlos syndrome
PXE
Porphyria cutanea tarda (not necessarily genetic form)
Werner syndrome
Rothmund Thompson
Cerebral amyloid angiopathy
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18
Q

Most patients with scleromyxoedema have plasma cell dyscrasia s with IgM and light chains are more commonly kappa

A

False - IgM and lambda

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19
Q

What are clinical features of scleromyxoedema ?

A

Numerous 2-3mm firm waxy closely aligned papules in a relatively symmetrical widespread pattern
- head and neck region
- upper trunk, hands, forearms and thigh
- papules often have a strikingly linear array
Surrounding skin shiny and indurated (sclerodermoid)
Glabella deep longitudinal furrowing
Shar-pei sign
Doughnut sign
Leonine facies

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20
Q

Scleromyxoedema is very rarely pruritic

A

False, pruritus not uncommon

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21
Q

Scleromyxoedema mucous membranes and scalp usually involved

A

False , spared

22
Q

30% cases of scleromyxoedema progress to systemic myeloma

A

False, <10%

23
Q

Dermato neuro syndrome of scleromyxoedema encompasses what features?

A

Convulsion, fever, coma, cardiovascular complications, haem malignancy

24
Q

Hep C is prevalent in scleromyxoedema

A

True - in Japanese patients (Lebwohl)

25
Q

Four types of lichen myxoedematosus

A 
PAIN HIV
Papular;
Acral;
Of infancy;
Nodular 
HIV associated
26
Q

Spontaneous resolution of lichen myxoedematosus can occur except in case of HIV associations

A

False - can occur even in setting of HIV infection

27
Q

Scleredema 3 types are?

A

1 - post infectious especially strep.
- face expressionless and opening mouth and swallowing can be difficult
2 - possible haem malignancy associated 25%
3 - diabetes mellitus associated

28
Q

Pretibial myxoedema can progress to pitting oedema

A

False - non pitting oedema and elephantiasis

29
Q

Pretibial myxoedema contains large amounts of mucin deposited in the papillary dermis

A

False RETICULAR dermis

30
Q

Natural history of pretibial myxoedema

A

May persist for life but can also clear spontaneously on average over 3.5 years

31
Q

Trauma can be the inciting factor for cutaneous focal mucinoses

A

True

32
Q

Primary follicular mucinosis can often resolve spontaneously after 1-2months

A

False 2-24 months

33
Q

What are mucocutaneous clues that suggest a possible nutritional disorder

A

Hair - sparse, flag sign (bands of light and dark discolouration), sparse hair, brittle hair, seb derm
Oral cavity - cheilitis, glossitis, gingival hypertrophy, bleeding Gums, mucosal erosions
Eyes - conjunctivitis, blepharitis
Photodistributed dermatitis with shellac like scale
Nails - koilonychia, slow and thin growth, pustular paronychia
Follicular hyperkeratosis and perifollicular haemorrhage, corkscrew hairs
ECCHYMOSES, petechiae, and poor wound healing

34
Q

Zinc deficiency inherited form - gene + inheritance?

A

SLC39A4, autosomal recessive, encodes ZIP4 transporter

35
Q

Maternal risk factors for zinc deficiency

A 
Alcoholism
Anorexia
Diet high in mineral binding phytate
Vegan diets
Intestinal malabsorption
Pregnancy, HIV infection, CRP, drugs (penicillamine)
36
Q

Supplementation for zinc deficiency is 1-2 mg/kg/day for acquired and 3mg/kg/day for life for acrodermatitis enteropathica

A

True

Rx zincsulfate more affordable than gluconate. 10mg/mL

37
Q

Stages of lipoid proteinosis

A

these occur after 2 years
1 - vesicles and crust that can resolve with ice pick scarring
Occurs on skin of face , mouth and extremities
2 - thickening of skin due to hyalin deposits within the dermis .
Verrucous lesions over extensors., alopecia eyebrows, lashes, corneal ulcers,

38
Q

Genetics of lipoid proteinosis

A

Loss of function mutations in ECM-1

39
Q

Types of colloid milium

A

Adult - multiple SYMMETRICAL yellow to flesh coloured facial papules assoc. with photo exposure
Nodular - flesh coloured nodule on face, scalp or chest usually solitary
Juvenile -translucent papules on cheeks, most , perioral skin, onset before puberty
Pigmented - variant of adult in which there is topical app of hydroquinone or phenol

40
Q

Nodular colloid milium can be associated with myeloma and has plasma cells

A

False, associated but without plasma cells

41
Q

Adult colloid milium is asymmetrical

A

False symmetrical

42
Q

Juvenile colloid milium not related to UV exposure

A

False - UV light induced damage has also been observed

43
Q

Colloid milium can be inherited

A

True , both autosomal dominant and recessive forms

44
Q

Colloid milium stains positive for Congo red and PAS

A

True , pan cytokeratin stain negative to help distinguish from primary cutaneous amyloidosis

45
Q

Factors predisposing to acute GVHD

A

HLA mismatch
Gender mismatch
Older recipient age >40y
Total body irradiation

46
Q

Factors predisposing to chronic GVHD - DONOR risk factors

A 
DONOR 
gender mismatch
Older recipient and donor 
Alloimmunisation eg. Prior pregnancy
Disparate HLA to recipient
47
Q

Factors predisposing to chronic GVHD - RECIPIENT risk factors

A

Non traditional conditioning regime, age >40y, prior acute GVHD, CML

48
Q

Factors predisposing to chronic GVHD - TRANSPLANT risk factors

A

Use of peripheral blood stem cells

T cell replete graft

49
Q

GVHD is only excluded on histo when there is a VERY high number of eos ie. 16 eos per 10 high power fields

A

True

50
Q

Skin staging of GVHD

A

Stage 1 - <25% BSA
Stage 2 - 25-50% BSA
Stage 3 - >50% to generalised erythroderma
Stage 4 - generalised erythroderma WITH bulla formation

Derm 2020 (59 decks)

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