2: Cutaneous Manifestations Of Systemic Disease and Other Skin Conditions Flashcards

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1
Q

What % of SLE cases have skin involvement

A

80

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2
Q

What are two classic rashes seen in SLE

A

Malar rash

Discoid rash

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3
Q

What is a malar rash

A

Rash over facial region with sparing of naso-labial folds

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4
Q

How will a discoid rash present

A

Erythematous patches with keratotic scaling

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5
Q

What may be present in the mouth in SLE

A

Oral Ulcers

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6
Q

What is a cutaneous manifestation of rheumatoid arthritis

A

Rheumatoid Nodules

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7
Q

How will systemic sclerosis present

A

thickening and hardening of the skin giving a smooth and shiny appearance

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8
Q

what are key features of the skin in systemic sclerosis

A
  • Thickened

- Shiny

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9
Q

how will pigmentation present in systemic sclerosis

A

Reduced pigmentation

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10
Q

what is a feature of the hands in systemic sclerosis

A

Sclerodactyly

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11
Q

what is sclerodactyly

A

Blue discolouration.

Oedema and fibrosis giving a waxy appearance to the skin with limited range of motion.

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12
Q

what is a classic facial feature of systemic sclerosis

A

Microstomia

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13
Q

what is a manifestation of limited cutaneous systemic sclerosis

A

CREST syndrome

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14
Q

what are the features of CREST syndrome

A
Calcinosis cutis 
Raynauds
Esophageal dysmotility 
Sclerodactyly
Telangectasoa
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15
Q

how can addison’s disease manifest cutaneously

A

pigmentation of regions not typically exposed to sunlight

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16
Q

why is there hyper-pigmentation in addison’s disease

A

due to increase in ACTH. Melanin is cleaved from the same precursor

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17
Q

What is a sign of dermatomyositis

A
  • Gottron’s Papules
  • Mid-Facial Rash
  • Heliotrope Rash
  • Photosensitive Poikiloderma
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18
Q

What are gottrons papules

A

Erythematous papules symmetrically distributed over extensor surface of the hand

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19
Q

What is a heliotrope rash

A

Erythematous rash on upper eye-lids accompanied by oedema

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20
Q

What is a mid-facial rash

A

Rash of mid face including naso-labial folds

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21
Q

What cutaneous manifestation is pathognomic of cutaneous sarcoidosis

A

Lupus pernio

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22
Q

What is lupus pernio

A

Purple papules on cheeks, nose, chin and ears

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23
Q

What is a cutaneous sign of diabetes mellitus

A

Acanthosis Nigricans

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24
Q

What is acanthosis nigricans

A

Hyperpigmentation of skin folds

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25
Q

How will neurfibromatosis I manifest cutaneously

A
  • Cafe Au Last Spots
  • Lisch nodules (hyperpigmentation of the iris)
  • Hyperpigmentation of axillary and inguinal nodes
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26
Q

How will tuberous sclerosis present clinically

A
  • Adenoma Sebaceum = red nodules in butterfly appearance around the nose
  • Shagreen patch = flesh-coloured papule of limbo-scral region
27
Q

What is erythema nodosum

A

inflammation of fat secondary to delayed hypersensitivity reaction

28
Q

What condition may erythema nodosum occur in

A

inflammatory bowel disease

29
Q

How will erythema nodosum present

A

painful subcutaneous nodules on pre-tibial surface

30
Q

If an intra-epidermal blister, are blisters more or less likely to rupture

A

more likely

31
Q

If an sub-epidermal blister, are blisters more or less likely to rupture

A

blister will form between the epidermis and dermis - therefore less likely to rupture

32
Q

What is bullous pemphigoid

A

autoimmune blistering skin condition

33
Q

What is the most common autoimmune blistering skin condition

A

bullous pemphigoid

34
Q

Which age-group does bullous pemphigoid tend to occur

A

> 60y

35
Q

what antibodies are present in bullous pemphigoid

A

anti-hemidesmasome

36
Q

what are 5 triggers of blistering in bullous pemphigoid

A
NSAIDs
Antbiotics
Furosemide
Catopril 
Penicillinamine
37
Q

how does bullous pemphigoid present clinically

A

Flaccid bullae over erythematous base common over TRUNK and LEGS. They are itchy. No mucosal involvement. Blisters heal without scarring.

38
Q

will bullous pemphigoid lesions heal with scarring

A

No

39
Q

what antibodies are present in bullous pemphigoid

A

Anti-hemidesmasome

40
Q

in which layers of the skin do blisters form in bullous pemphigoid

A

Sub-epidermal

41
Q

what type of hypersensitivity reaction is bullous pemphigoid

A

Type II

42
Q

what mediates type II hypersensitivity reactions

A

Antibodies (IgG and IgM)

43
Q

What are the two anti hemi-desmasome antibodies

A

BP180 and BP230

44
Q

What two signs are negative in bullous pemphigoid

A

Tzanck

Nikoloskys

45
Q

What is Tzanck Test

A

Deroof and scrape vesicle. Scraping is put on a glass slide and giemsa stained.

46
Q

What is a negative Tzanck test

A

No multinucleate cells on gram-staining

47
Q

What is nikoloskys sign

A

formation of blister on stroke skin

48
Q

What may be ordered for bulbous pemphigoid

A

Skin histology and immunohistochemistry

49
Q

What can be seen on histology in bullous pemphigoid

A

Deposition of IgG and C3 along sub-epidermal junction

50
Q

What is used to manage localised bullous pemphigoid

A
  • Wound dressings

- Topical corticosteroids

51
Q

What is used to manage wide spread bullous pemphigoid

A

Oral corticosteroids

52
Q

What is pemphigus vulgaris

A

autoimmune skin condition caused by IgG raised to desmoglein-3

53
Q

What age is pemphigus vulgaris common in

A

40-60

54
Q

What ethnicity is pemphigus vulgaris common in

A

Ashkenazi Jews

55
Q

What antibodies are present in pemphigus vulgaris

A

IgG raised to desmoglein-3

56
Q

What may trigger pemphigus vulgaris

A
  • Medications
  • Diet
  • UV
57
Q

What three drugs can trigger pemphigus vulgarise

A

ACEi
Penicillamine
Phenolbarbitone

58
Q

Where do lesions in pemphigus vulgaris originate

A

Mucosa

59
Q

Where will lesions in pemphigus vulgaris then present

A

Intertriginous areas = regions of high-pressure

60
Q

Explain the four-stages of lesions in pemphigus vulgaris

A
  1. Flaccid bullae form and then rupture
  2. Ruptured lesions then become confluent
  3. Ruptured lesions crust over
  4. Hyperpigmentation as a result
61
Q

What type of blisters occur in pemphigus vulgaris

A

Intra-epidermal

62
Q

What signs are positive in pemphigus vulgaris

A

Nikolsky

Tzanck

63
Q

On histology and immunohistochemistry what will be seen in pemphigus vulgaris

A

Antibodies raised to IgG

64
Q

What is used to manage pemphigus vulgaris

A

Wound dressing

High-dose corticosteroids