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

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
How will neurfibromatosis I manifest cutaneously
- Cafe Au Last Spots - Lisch nodules (hyperpigmentation of the iris) - Hyperpigmentation of axillary and inguinal nodes
26
How will tuberous sclerosis present clinically
- Adenoma Sebaceum = red nodules in butterfly appearance around the nose - Shagreen patch = flesh-coloured papule of limbo-scral region
27
What is erythema nodosum
inflammation of fat secondary to delayed hypersensitivity reaction
28
What condition may erythema nodosum occur in
inflammatory bowel disease
29
How will erythema nodosum present
painful subcutaneous nodules on pre-tibial surface
30
If an intra-epidermal blister, are blisters more or less likely to rupture
more likely
31
If an sub-epidermal blister, are blisters more or less likely to rupture
blister will form between the epidermis and dermis - therefore less likely to rupture
32
What is bullous pemphigoid
autoimmune blistering skin condition
33
What is the most common autoimmune blistering skin condition
bullous pemphigoid
34
Which age-group does bullous pemphigoid tend to occur
>60y
35
what antibodies are present in bullous pemphigoid
anti-hemidesmasome
36
what are 5 triggers of blistering in bullous pemphigoid
``` NSAIDs Antbiotics Furosemide Catopril Penicillinamine ```
37
how does bullous pemphigoid present clinically
Flaccid bullae over erythematous base common over TRUNK and LEGS. They are itchy. No mucosal involvement. Blisters heal without scarring.
38
will bullous pemphigoid lesions heal with scarring
No
39
what antibodies are present in bullous pemphigoid
Anti-hemidesmasome
40
in which layers of the skin do blisters form in bullous pemphigoid
Sub-epidermal
41
what type of hypersensitivity reaction is bullous pemphigoid
Type II
42
what mediates type II hypersensitivity reactions
Antibodies (IgG and IgM)
43
What are the two anti hemi-desmasome antibodies
BP180 and BP230
44
What two signs are negative in bullous pemphigoid
Tzanck | Nikoloskys
45
What is Tzanck Test
Deroof and scrape vesicle. Scraping is put on a glass slide and giemsa stained.
46
What is a negative Tzanck test
No multinucleate cells on gram-staining
47
What is nikoloskys sign
formation of blister on stroke skin
48
What may be ordered for bulbous pemphigoid
Skin histology and immunohistochemistry
49
What can be seen on histology in bullous pemphigoid
Deposition of IgG and C3 along sub-epidermal junction
50
What is used to manage localised bullous pemphigoid
- Wound dressings | - Topical corticosteroids
51
What is used to manage wide spread bullous pemphigoid
Oral corticosteroids
52
What is pemphigus vulgaris
autoimmune skin condition caused by IgG raised to desmoglein-3
53
What age is pemphigus vulgaris common in
40-60
54
What ethnicity is pemphigus vulgaris common in
Ashkenazi Jews
55
What antibodies are present in pemphigus vulgaris
IgG raised to desmoglein-3
56
What may trigger pemphigus vulgaris
- Medications - Diet - UV
57
What three drugs can trigger pemphigus vulgarise
ACEi Penicillamine Phenolbarbitone
58
Where do lesions in pemphigus vulgaris originate
Mucosa
59
Where will lesions in pemphigus vulgaris then present
Intertriginous areas = regions of high-pressure
60
Explain the four-stages of lesions in pemphigus vulgaris
1. Flaccid bullae form and then rupture 2. Ruptured lesions then become confluent 3. Ruptured lesions crust over 4. Hyperpigmentation as a result
61
What type of blisters occur in pemphigus vulgaris
Intra-epidermal
62
What signs are positive in pemphigus vulgaris
Nikolsky | Tzanck
63
On histology and immunohistochemistry what will be seen in pemphigus vulgaris
Antibodies raised to IgG
64
What is used to manage pemphigus vulgaris
Wound dressing | High-dose corticosteroids