Blisters and emergencies Flashcards

1
Q

Describe what is meant by bullous pemphigoid [1]

It is caused by antibodies agaisnt which basement membrane proteins? [2]

Which patients is it commonly found in?

A

In frail patients
Autoimmune blistering condition
Antibodies against basement membrane proteins which are key components of hemidesmosomes
* BP 180
* BP 230

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2
Q

Which neurological conditions is Bullous Pemphigoid associated with? [3]

Which medications are Bullous Pemphigoid associated with? [2]

A

Neurological disease (that cause frailty)
* Stroke
* Dementia
* Parkinson’s Disease

Drugs:
* PD-1 Inhibitors (used in the treatment of metastatic melanoma)
* Gliptins (diabetic medication)

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3
Q

Describe the clinical manifestations of Bullous Pemphigoid [5]

A
  • Severe itch
  • Pre-bullous rash
  • Large tense bullae and erosions on rupture
  • Milia in healed areas
  • Multple, large tense blisters - that leave eriosions after they burst
  • Mucosal lesions uncommon

NB: top right shows the erosion after a blister has gone

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4
Q

What is a classic differentiating feature between pemphigoid and pemphigus? [2]

A

Bullous pemphigoid:
- mouth is usually spared
- blisters are more tense

Pemphigus vulgaris:
- Mouth commonly involved
- blisters commonly burst

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5
Q

Describe the topical treatments [4] and systemic therapy [5] used to treat Bullous Pemphigoid

A

Topical Treatments
* Super potent topical steroid
* Non adhesive dressings
* Potassium per manganate soaks
* Pop large blisters

Systemic therapy
* Antihistamines
* Tetracycline antibiotic vs oral steroid (BLISTER trial)
* Methotrexate
* Mycophenolate Mofetil
* Biologic therapy - Rituxumab

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6
Q

Describe how you investigate for bullous pemphigoid

A

Punch biopsy:
- immunofluorescence shows IgG and C3 at the dermoepidermal junction

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7
Q

Complications associated with BP? [4]

A

Secondary infections:
* Staphylococcus aureus and Pseudomonas aeruginosa are the most common organisms
* risk of sepsis is significant due to disrupted skin integrity

Side effects from treatment:
- significant corticosteroid use: osteoporosis, diabetes mellitus, hypertension, glaucoma or cataracts

Elderly-related complications::
- Cognitive impairment has been linked with bullous pemphigoid

Increased risk of cardiovascular disease and stroke

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8
Q

Young person x Erosions x Mucosal ulceration =

A

Pemphigus Vulgaris

NB: erosions are previous flaccid blisters

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9
Q

Describe the pathophysiology of Pemphigus Vulgaris [1]

A

Autoimmune blistering condition
- Autoantibodies to Desmoglein-3 (and sometimes also Desmoglein-1) which are found in desmosomes
- The autoantibodies belong to the IgG class, specifically IgG4

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10
Q

Describe the clinical features of pemphigus vulgaris [+]

A
  • Mucosal involvement usually precedes skin involvement – mouth + genitals: oral lesions are common and often present as the initial clinical feature.
  • Thin-walled flaccid blisters filled with clear fluid
  • Rupture easily
  • Itchy, painful erosions
  • Painful ! :( - epidermis has gone and exposing the dermis
  • Often lose weight (due to mouth ulcers stopping eating)
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11
Q

Which drugs can induce PV? [2]

A

Penicillamine
ACE-inhibitors

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12
Q

PV can be a pareneoplastic disease of which cancers? [2]

A

Lymphomas
Castleman disease

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13
Q

Dx of Pemphigus Vulgaris ?

A

Punch/incisional biopsy of:
* Lesional skin for H&E
* Peri-lesional skin direct immunofluorescence
- Fishnet pattern of intercellular IgG and C3 deposits within the epidermis

Serum Autoantibody Testing:
- test measures circulating autoantibodies against desmoglein 1 and 3

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14
Q

Tx of Pemphigus Vulgaris? [4]

A

First-Line Treatment
- oral corticosteroid ± azathioprine or mycophenolate

Second line:
- Rituximab

Intravenous immunoglobulin (IVIG) and plasmapheresis are adjunctive therapies for severe or refractory cases.

Patients on high-dose steroids or other immunosuppressants should receive prophylaxis against Pneumocystis jirovecii pneumonia with co-trimoxazole.

Notes: BMJ BP

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15
Q

Dx? [1]

A

Dermatitis Herpetiformis

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16
Q

Clinical features of dermatitis herpetformis? [5]

A

Symmetrical distribution

Scalp, shoulders, buttocks, elbows, knees

Very itchy papules and vesicles: scratching often leads to excoriation and crusting

onset of symptoms is variable and can occur at any age, although it is most common in the third to fifth decades of life

The disease course is characterized by periods of exacerbation and remission.

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17
Q

How do you dx dermatitis hepetiformis? [3]

A

Clinical presentation

Serology:
- Elevated levels of anti-tissue transglutaminase (tTG) antibodies or endomysial antibodies (EMA). Anti-deamidated gliadin peptide (DGP) antibodies may also be elevated.

Small intestinal biopsy

Histopathology:
- demonstrating subepidermal blisters with neutrophilic infiltrate

Response to diet:
- Improvement of symptoms upon adherence to a strict gluten-free diet is supportive but not solely diagnostic.

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18
Q

Tx for Dermatitis Herpetiformis? [2]

A

Gluten free diet for life
Dapsone

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19
Q

Dapsone can be used to treat dermatitis herpetiformis.

Name some side effects of using as treatment [2]

A

hemolytic anaemia and methemoglobinemia

  • regular monitoring of complete blood counts and methemoglobin levels.
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20
Q

Patients with dermatitis herpetiformis have an increased risk of developing [], a rare but aggressive form of non-Hodgkin lymphoma.

A

Patients with dermatitis herpetiformis have an increased risk of developing Enteropathy-associated T-cell lymphoma (EATL), a rare but aggressive form of non-Hodgkin lymphoma.

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21
Q

Define erythroderma [1]

Name 5 causes

A

Widespread erythema of skin (over 90% of body surface area)

Causes
* Idiopathic (30%)
* Adverse drug reaction (gold)
* Known inflammatory skin disease (eczema, psoriasis)
* Cutaneous lymphoma

22
Q

Explain some complications of erythroderma

A

Secondary infection
Acute renal failure
- water loss from skin; immune deposits in kidney

High output cardiac failure

23
Q

Mx of Erythroderma? [6]

A

Stop unnecessary or suspect drugs
Appy greasy emollients every 2 hours (50:50)
Take swabs from oozing skin (microbiology)
Antihistamines
Treat secondary infection
Fluids / Monitor U+Es

24
Q

Dx? [1]
Describe the pattern seen here [1]

A

Eczema Herpeticum
* Monomorphic crusts / vesicles / papules
* History of eczema

25
Describe how eczema herpeticum occurs [2]
**Atopic dermatitis** results in **impaired skin protective function**, allowing **easier infection of HSV***Disruption of the epidermal barrier is the most important risk factor* **Inflammation and immune dysregulation** may allow for **widespread infection of the virus** HSV requires intact cellular immunity to be contained
26
Describe the clinical presentation of eczema herpticum [6]
The skin lesions of eczema herpeticum typically present as **painful clusters of blisters, fluid-filled elevations of the skin** **Areas of rapidly worsening, painful eczema** The blisters appear widely over the body but are **most common on the face, neck, and trunk**. **Older blisters** that have **burst** and **dried** commonly form **“punched out” erosions**, which are **circular breaks in the continuity** of the skin that may **bleed or produce pus.** Possible **fever, lethargy, lymphadenopathy or distress**
27
What is meant by 'eczema herpeticum incognito'? [1]
presentation of EH that is easily **mistaken for impetigo** and most often seen in patients with severe AD and recurrent EH
28
What is the name for when HSV can spread to the eye? [1] What is the management? [1]
Rarely, HSV may spread to the eye resulting in **herpes keratitis** **Ganciclovir ointment five times daily** (3 hourly) for 7-10 days
29
Tx for Eczema Herpeticum? [2]
Acyclovir Flucoxacillin
30
Describe what is meant by erythema multiforme
**Erythema multiforme (EM) is a skin hypersensitivity reaction that commonly occurs secondary to an infection or drugs.*** A hypersensitivity reaction is an exaggerated immune reaction that occurs in response to an antigen or allergen.*
31
What commonly causes erytherma multiforme? [3] Describe the two types (EM minor/major) [2]
**Causes**: **Drugs** or Infections (**HSV, Mycoplasma**) **EM Minor** – target lesions + mild/ no mucosal involvement **EM Major** – target lesions + 1 or more mucosal surfaces involved
32
Which areas are commonly affected by EM?
**Symmetrical** **distribution** on the **extensor surfaces of acral extremities** **Mucosal lesions** commonly involve the **oral** **mucosa** and can lead to **erythema, erosions, and/or bullae.**
33
Describe the lesions in EM [3] Where else can be commonly effected? [1]
EM is characterised by the presence of target lesions that are usually < 3 cm in size. These lesions have three striking features: * **A central blister or dusky red area** * **A surrounding pale ring of oedema** * **An erythematous halo in the periphery** It does not usually affect the mucous membranes but can cause a sore mouth (**stomatitis**).
34
A patient takes penicillin for an infection. They come out in targetoid lesions. What type of hypersensitivty reaction is this? Type 1 Type 2 Type 3 Type 4
Type 1 Type 2 Type 3 **Type 4** EM is considered a cell-mediated (type IV) hypersensitivity reaction, which is an abnormal cell-mediated reaction initiated by T cells. The exact mechanism leading to EM is incompletely understood, but it appears to be an abnormal immune reaction to a drug metabolite or viral antigen that becomes expressed by keratinocytes within the skin
35
Why might you consider performing a CXR for unknown cause of EM? [1]
Where there is no clear underlying cause it may be worth investigating further, for example doing a **chest xray to look for mycoplasma pneumonia.**
36
What is the management for EM? [3]
Management * EM typically develops over 3-5 days and improves over 2 weeks. Some patients may get persistent or recurrent episodes. * Stop the drug * Treat the underlying infection * Treat with a week of **oral** **prednisolone** if severe mucosal pain/ difficulty eating
37
Dx? [1]
**SJS**
38
Describe what is meant by SJS [1] Describe the presentation of SJS [3]
**Stevens-Johnson syndrome** is a severe systemic reaction affecting the **skin and mucosa** that is almost always caused by a **drug reaction**. **Presentation**: * **Patients** usually start with non-specific symptoms of **fever, cough, sore throat, sore mouth, sore eyes and itchy skin**. * They then develop a **purple or red rash** that **spreads across the skin and starts to blister**. * **Nikolsky sign is positive** in erythematous areas - blisters and erosions appear when the skin is rubbed gently ## Footnote **NB**: Nikolsky sign = where very gentle rubbing of the skin causes it to peel away
39
Which drugs cause SJS? [7] Which infection can also can? [1]
**SPANLOC** * **S**ulphonamide * **P**enicillin * **P**henytoin * **Al**lopurinol * **N**SAIDS * **L**amotrigine * **O**CP * **C**arbamazepine Most commonly caused by drugs. Less commonly infection eg **Mycoplasma**
40
SJS vs TEN? [1]
SJS < 10% body surface area TEN > 30% body surface area
41
Describe in detail the skin lesions seen in SJS / TENs [4]
* Erythematous or purpuric macules * Diffuse erythema * Targetoid/ atypical target lesions * Flaccid blisters
42
How do you dx SJS / TEN? [1] What score is used for to classifiy the severity of SJS / TEN? [1]
**Dx**: - **skin biopsy** Severity: - **SCORTEN** ## Footnote sum of five points or more indicates a mortality risk of over 90%
43
Pathophysiology of staphylococcal scalded skin syndrome (SSSS)? [1]
**staphylococcus** **aureus** bacteria that produces **epidermolytic** toxins. These toxins are **protease enzymes** that break down the **proteins that hold skin cells together** When a skin infection occurs and these toxins are produced, the skin is damaged and breaks down. This condition usually affects children under 5 years. Older children and adults have usually developed immunity to the epidermolytic toxins.
44
Clinical presentation of SSSS? [5]
**Fever**: - High fever (>38.9°C) **Rash**: - A **diffuse**, **blanching** **erythroderma** resembling **sunburn** usually appears early in the disease course. - **Desquamation**, particularly on the **palms and soles**, typically occurs **one to two weeks after the onset of illness** **Myalgias and arthralgias** are frequently reported; **Hypotension**: - Hypotension or shock may be present, often manifesting as systolic blood pressure less than 90 mmHg.
45
Management for SSSS? [1]
* Admit * Fluids * **IV flucloxacillin** and **clindamycin** *Clindamycin suppresses toxin production, while flucloxacillin targets the bacterial cell wall.* * If methicillin-resistant Staphylococcus aureus (MRSA) is suspected, **vancomycin** or **daptomycin** can replace flucloxacillin.
46
What are the four types / causes of necrotising fasciitis? [4]
**Type 1** – Polymicrobial (anaerobes + aerobes) **Type 2** – Group A beta-haemolytic streptococci **Type 3** – Clostridium perfringens; also associated with Vibrio species infection from seafood ingestion or water contamination of wounds. **Type 4** - Fungal infection
47
EM
48
Which cause of NF can cause toxic shock syndrome? [1]
Type 2
49
**Lichen planus**
50
Lichen planus
51
**Co-amoxiclav**