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Derm 2020 > Autoimmune Connective Tissue Disorders > Flashcards

Autoimmune Connective Tissue Disorders Flashcards

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Dermatology Board Prep flashcards
1
Q

SLE can have a particulate ANA pattern

A

True - so can MCTD

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2
Q

Nuceolar pattern of ANA corresponds with SLE

A

False - scleroderma

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3
Q

SLE can have a homogeneous or rim ANA pattern

A

True

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4
Q

Neonatal LE is a variant of SCLE

A

True

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5
Q

List diseases associated with SCLE

A 
-	Associated diseases:
o	Sjogren syndrome
o	RA
o	Sweet syndrome
o	Crohn disease
o	LP
o	Psoriasis
o	Hereditary angioedema
o	PCT
o	GSE
o	TEN
o	Morphoea and dystrophic calcinosis cutis have sometimes followed presentation of SCLE
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6
Q

List cutaneous features of antiphospholipid syndrome

A 
-	Cutaneous features:
o	Livedo reticularis
o	Nail changes – nail fold ulcers
o	Vasculopathy
o	Anetoderma
o	Sneddon’s , superficial thrombophlebitis  migrans
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7
Q

Acute cutaneous LE is associated with usually fixed lesions

A

False - usually transient

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8
Q

SCLE induced by ..?

A

induced by hydrochlorothiazide, TNF-a inhibitors, terbinafine, antiepileptics and PPI

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9
Q

Dyspigmentation a common consequence of SCLE

A

True

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10
Q

SCLE can be annular (1/3), polycyclic (2/3) but do not demonstrate bullae or vesicles

A

False - can demonstrate vesiculation, crusting and occasional bullae along the borders

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11
Q

SCLE can leave telangiectases

A

True - no scarring but can leave hypopigmentation and telangiectases

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12
Q

Compare SCLE Histo to DLE

A

SCLE has more epidermal atrophy,
Less BM thickening, hyperkeratosis, follicular plugging and inflammation
Colloid bodies and epidermal necrosis in >50% of cases

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13
Q

Midfacial skin is usually involved in SCLE

A

False- spared

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14
Q

Acute cutaneous lupus spares the nasolabial fold

A

True

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15
Q

Presence of small erosions can aid in the clinical differential diagnosis, favouring acute cutaneous lupus

A

True

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16
Q

In lupus panniculitis , tissues are swollen, brawny, warm and tense

A

False - this is tumid lupus

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17
Q

Tumid lupus occurs most commonly on the upper and lower limbs

A

False - face and torso

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18
Q

Jessner’s lymphocytic infiltrate is morphologically similar to tumid lupus

A

True

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19
Q

Tumid lupus has a high prevalence of SLE

A

False - low prevalence ,with relative lack of serological abnormalities

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20
Q

Lupus panniculitis - does not occur in children

A

False - can occur in children in association with NLE and partial C4 deficiency

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21
Q

Lupus panniculitis more common in males

A

False - females

22
Q

Discoid lupus lesions can overlie lupus panniculitis

A

True

23
Q

Lupus panniculitis is a septal panniculitis

A

False - lobular

24
Q

Lupus panniculitis comprise non-tender subcutaneous nodules and plaques arising in crops

A

False - tender , rest is true

25
Q

Lupus panniculitis often occurs in lower extremities

A

False - unlike erythema nodosum

26
Q

Lupus panniculitis can present with lipoatrophy or induration

A

True

27
Q

Chilblain lupus shows tuft resorption on x-ray

A

True

28
Q

Chilblain lupus - fingers often show curious atrophic swindling sometimes with hyperextended terminal phalanges and nail dystrophy

A

True

29
Q

Chilblain lupus - 5% develop SLE

A

False - 15%

30
Q

NLE develops in the first few days of birth

A

True, but can also present up to 12-16 wk post partum

31
Q

What are the 3 types of hepatobiliary involvement with NLE?

A

1 . Fulminant liver failure presenting at or shortly after birth

  1. Cholestasis a few weeks after birth
  2. Transient mild to moderate transaminase elevations a few wk or months after birth
32
Q

How to monitor LFT for NLE

A

At diagnosis, then every 3 months until 9 months old

33
Q

What is criteria for bullous lupus ?

A
  1. Fulfils criteria for SLE according to ARA criteria
  2. Histo reminiscent of DH
  3. Bullous lesions in photodistributed areas
  4. linear or granular deposits of IgG +/- IgA +/- C3 at DEJ and immune reactants below the basal lamina by electron microscopy
34
Q

Blistering in bullous lupus often parallels systemic flares of SLE particularly affecting the kidneys

A

True

35
Q

Bullous lupus response to dapsone is often very gradual

A

False = response can be dramatic with bullae ceasing after 1-2 days

36
Q

Neonatal lupus erythematosus can present as a blueberry muffin baby

A

True

37
Q

List complications of NLE

A
  1. Cardiac mortality 20% and 2/3 need PPM
  2. DIC
  3. Scarring/teles
  4. Development of AI CT later in life
  5. Cardiovascular problems later in life
  6. Risk in subsequent pregnancy 25%
  7. Risk of NLE in anti-SSA and women 2%

F/U need repeat LFT every 3 mo until 9 mo old

38
Q

Drugs that induce dermatomyositis

A 
CHILL TNFa
Cyclophosphamide
Hydroxyurea
Ipilimumab (checkpoint inhibitors)
Lipid lowering agents - statins>gemfibrozil
Losec (PPIs)
TNF-a inhibitors
39
Q

Risk of malignancy with adult dermatomyositis

A

15-25%

40
Q

Infections that can induce dermatomyositis

A

E. Coli
Echovirus
Picornavirus
Cocksackie virus

41
Q

What are features of Anti-Jo1 DM

A 
High incidence of ILD
Raynaud phenomenon
Mechanics hands
EROSIVE POLYARTHRITIS
PROXIMAL MUSCLE WEAKNESS
Antisynthetase syndrome
42
Q

Features of Anti-TIFy

A

Malignancy associated myositis in adult disease,
extensive cutaneous disease in adult or juvenile DM,
Palmar hyperkeratotic papules
Psoriasiform lesions
Red on white telangiectatic patches
Ovoid palatial patch

43
Q

Anti-NXP2

A

Malignancy associated myositis
Adult and juvenile DM with calcinosis , peripheral oedema, myalgia, dysphagia
Mild skin disease

44
Q

Anti-SAE1

A

Interstitial Lung disease and dysphagia

Amyopathic dermatomyositis

45
Q

Anti-Mi2

A

Milder muscle disease
Cutaneous disease
Classic Gottron/Shawl/Ragged cuticles
Adult or juvenile DM

46
Q

Anti-MDA5/CADM-40

A

Amyopathic DM

Cutaneous ulcerations - periungal region and overlying Gottron papules and Gottron sign

47
Q

Anti-SRP (signal recognition protein)

A

Acute onset necrotising myopathy (severe weakness, high CK0, which may be refractory to treatment.
Incr cardiac mortality

48
Q

How does limited SSc differ from diffuse SSc

A

Limited SSc - CREST - more gradual progression and longstanding Raynaud phenomenon

  • later onset of internal organ involvement
  • PAH more common/ILD less common
  • more favourable long term prognosis

Diffuse SSc

  • sudden onset of Raynaud’s phenomenon
  • Rapidly progressive , more widespread cutaneous sclerosis. Usually peaks within 12-18 months
  • > 90% have internal organ involvement within the first 5 years
  • ILD>PAH
  • kidney disease
49
Q

Telangiectases are associated with the severity of vasculopathy in systemic sclerosis

A

True (JAAD July 2018 article)

50
Q

What are findings on respiratory function test in SSc

A

Decreased FEV1,
decr FVC but incr ratio FEV1/FVC due to decline in FVC>decline in FEV1 , Ratio >80%
Decreased DLCO

51
Q

What are differentials for scleroderma ?

A

MENSES TOXIN GVHD GENE

  • morphoea
  • eosinophilic fasciitis
  • nephrogenic systemic fibrosis
  • scleromyxoedema
  • endocrine: diabetic cheiroarthropathy, PCT
  • scleroderma

TOXINS: silicosis, vinyl chloride, toxic oil, gadolinium, bleomycin, taxanes

GVHD

Genetic: restrictive dermopathy , Werner syndrome, Hutchinson-Gilford/ progeria , stiff skin syndrome, pachydermoperiostosis, ataxia-telangiectasia, Huriez syndrome

Derm 2020 (59 decks)

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