Blistering Skin Disease Flashcards

(126 cards)

1
Q

What is blistering skin disease?

A

A condition characterised by fluid filled lesions

Blistering skin diseases can be caused by various factors, including infections, autoimmune disorders, and genetic conditions.

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2
Q

How can blistering skin disease present?

A

May be localised or generalised

Localised forms affect specific areas, while generalised forms can cover large portions of the body.

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3
Q

What is the severity range of blistering skin disease?

A

Can vary from benign to life-threatening

The impact of the disease depends on its underlying cause and extent of lesions.

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4
Q

What is acantholysis?

A

The loss of adhesion between the keratinocytes in the epidermis.

Acantholysis can lead to various skin disorders.

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5
Q

Define spongiosis.

A

The widening of the intercellular spaces between keratinocytes due to intercellular edema that occurs when the epidermis is inflamed.

Spongiosis is commonly seen in conditions like eczema.

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6
Q

What does dissociation refer to in the context of skin layers?

A

The loss of adhesion between the epidermis and the dermis.

This can lead to blister formation and other skin pathologies.

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7
Q

On what factor does the diagnosis of blistering dermatoses depend?

A

The site of the intracellular split within the epidermis

This indicates that the location of the cleavage in the skin layers plays a crucial role in determining the specific type of blistering dermatosis.

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8
Q

What are the skin cleavage levels in blistering dermatoses?

A
  • Subcorneal
  • Intraepidermal
  • Subepidermal

Each level corresponds to different types of blistering conditions based on where the split occurs.

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9
Q

What are examples of subcorneal skin cleavage conditions?

A
  • Bullous impetigo
  • Staphylococcal scalded skin syndrome

These conditions are characterized by blisters forming just below the outermost layer of the skin.

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10
Q

What are examples of intraepidermal skin cleavage conditions?

A
  • Pemphigus vulgaris
  • Pemphigus foliaceus
  • Varicella
  • HSV

Intraepidermal conditions involve splits within the epidermis itself.

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11
Q

What are examples of subepidermal skin cleavage conditions?

A
  • Bullous pemphigoid
  • Dermatitis herpetiformis
  • Toxic epidermal necrolysis

These conditions occur when the cleavage happens below the epidermis.

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12
Q

What characterizes localized blistering skin diseases?

A

Localized to 1 body site and arises due to infection or inflammatory disorders

These conditions typically do not spread throughout the body and can often be treated effectively.

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13
Q

What are some examples of localized blistering skin diseases?

A
  • Enteroviral infection (hand-foot-and-mouth disease)
  • Herpes simplex
  • Herpes zoster
  • Acute dermatitis
  • Fixed drug eruption
  • Bullous impetigo

These conditions are typically less severe and limited to specific areas.

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14
Q

What characterizes generalized blistering skin diseases?

A

Can be life-threatening and may require hospital admission

Generalized conditions spread across the body and often indicate serious underlying health issues.

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15
Q

What are some examples of generalized blistering skin diseases?

A
  • Atypical enteroviral infection
  • Varicella
  • Eczema herpeticum
  • Erythema multiforme
  • Pemphigoid
  • Pemphigus
  • Stevens-Johnson syndrome or toxic epidermal necrolysis
  • Inherited blistering diseases

Generalized conditions often require more intensive medical care.

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16
Q

True or False: Localized blistering skin diseases can arise from infection or inflammatory disorders.

A

True

Localized diseases are often triggered by specific infections or localized inflammatory responses.

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17
Q

Fill in the blank: Acute generalized blistering skin disease can be __________ and may require hospital admission.

A

[life-threatening]

This highlights the severity and potential complications associated with generalized blistering conditions.

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18
Q

What develops beneath the stratum corneum of the epidermis as a result of acantholysis?

A

Subcorneal bullae

Acantholysis refers to the loss of connections between keratinocytes, leading to the formation of blisters.

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19
Q

Describe the characteristics of subcorneal bullae.

A

Flaccid and have a very thin roof that easily ruptures

This makes them prone to breaking under minimal pressure.

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20
Q

Where do intraepidermal bullae form?

A

Within or between keratinocytes in the stratum spinosum and stratum granulosum layers of the epidermis

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21
Q

What is the process called that leads to the formation of intraepidermal bullae?

A

Spongiosis

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22
Q

What characteristic do the roofs of intraepidermal bullae have?

A

Thin roof that easily ruptures

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23
Q

What are subepidermal blisters?

A

Blisters that occur within the basement membrane zone as a result of separation between the epidermis and the dermis

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24
Q

What is the process that leads to the formation of subepidermal blisters?

A

Dissociation

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25
How would you describe the bullae associated with subepidermal blisters?
Tense and do not easily rupture
26
What are the types of distribution for cutaneous blisters?
- Generalized - Grouped - Dependent areas - Hands or feet - Photodistributed - Dermatomal - Linear
27
What is bullous pemphigoid?
The most common form of autoimmune subepidermal blistering disease.
28
In which patient population is bullous pemphigoid more prevalent?
Older patients with neurological diseases such as dementia, stroke, or Parkinson's disease.
29
List some drugs that may trigger bullous pemphigoid.
* Diuretics * Nonsteroidal anti-inflammatories (NSAIDs) * Angiotensin-converting enzyme (ACE) inhibitors * Antibiotics * TNF alpha inhibitors * DPP-4 inhibitors
30
Where does bullous pemphigoid typically involve?
The flexor aspects of the limbs.
31
What are the characteristics of bullous pemphigoid lesions?
* Severe itch * Large, tense bullae that rupture to form crusted erosions
32
What are some features that may appear before vesicles or blisters in bullous pemphigoid?
* Nonspecific rash for several weeks * Eczematous areas * Urticaria-like red skin * Annular lesions
33
What is generalized bullous pemphigoid?
The most common form of bullous pemphigoid that can become widespread within days.
34
What is localized bullous pemphigoid?
A less common form usually limited to the lower extremities.
35
What is the treatment approach for localized bullous pemphigoid?
May be treated at the primary care level.
36
What is epidermolysis bullosa acquisita (EBA)?
A rare autoimmune blistering disease characterized by the formation of tense subepithelial blisters.
37
What is the most common form of EBA?
Classical mechanobullous, with localized, tense vesicles at easily injured sites.
38
Describe the non-classical form of EBA.
Widespread involvement with vesicles not localized to sites of trauma, often affecting the trunk and skin folds.
39
What is dermatitis herpetiformis linked to?
Celiac disease, a gluten-sensitive enteropathy.
40
What age group predominantly experiences dermatitis herpetiformis?
Caucasians aged between 15 and 40.
41
What are the common locations for lesions in dermatitis herpetiformis?
* Scalp * Shoulders * Buttocks * Elbows * Knees
42
What characterizes pemphigus vulgaris?
Development of blisters on mucous membranes and subsequently on the skin.
43
What age group is most commonly affected by pemphigus vulgaris?
Individuals between the ages of 30 and 60.
44
What are the typical appearances of skin lesions in pemphigus vulgaris?
Thin-walled flaccid blisters filled with clear fluid that easily rupture.
45
What type of testing is used for diagnosing skin conditions?
Skin biopsy (Bx).
46
What does direct immunofluorescence show in bullous pemphigoid?
Fine, linear deposits of IgG and/or C3 along the epidermal basement membrane.
47
What is the first-line treatment for mild and/or localized bullous pemphigoid?
Superpotent topical corticosteroids.
48
What is the second-line treatment for extensive and/or persistent bullous pemphigoid?
Urgent referral to dermatology to assess and treat.
49
What are the second-line treatments for mild and/or localized bullous pemphigoid?
* Oral corticosteroids * Minocycline, doxycycline, or tetracycline with nicotinamide * Erythromycin, penicillins * Dapsone, sulphonamides * Topical immunomodulatory (e.g., tacrolimus)
50
What general measures should be taken for bullous pemphigoid treatment?
Use non-adhesive dressings on broken skin, leave blisters intact if possible, burst blisters with a sterile needle if necessary, and keep a patient blister diary.
51
What is the purpose of a patient blister diary?
To record the number and location of blisters appearing daily, helping to monitor the response to treatment.
52
What topical therapy is recommended for bullous pemphigoid?
Apply topical dermovate ointment and use an emollient such as paraffin gel.
53
What is the recommended systemic therapy for bullous pemphigoid?
Oral prednisolone 0.5 - 1mg/kg/day, oral tetracycline antibiotics like doxycycline or minocycline, and nicotinamide 500mg BD.
54
True or False: Bullous pemphigoid is a benign condition that does not lead to complications.
False
55
What complications can arise from bullous pemphigoid?
Increased morbidity and mortality, secondary infections, and risks from systemic steroid use.
56
What types of infections can occur in sites of broken skin in bullous pemphigoid?
Bacterial (staphylococcal and streptococcal) and viral infections (herpes simplex, varicella, herpes zoster).
57
What are potential risks associated with long-term steroid use?
Infections, hypertension, diabetes, and osteoporosis.
58
What baseline routine blood tests are required for patients on long-term steroids?
Full blood count, urea and electrolytes, liver function test, calcium and vitamin D level check.
59
What tool is used to assess the risk of osteoporosis in patients on long-term steroids?
Fracture risk assessment (FRAX) tool.
60
What supplementation might be considered for patients on systemic steroids?
Calcium and vitamin D supplementation.
61
Fill in the blank: The optimum duration of treatment for bullous pemphigoid has not been established, but most patients need to be treated for at least ______ months.
6–12
62
What gastric protection may be considered for patients on long-term systemic steroids?
A proton pump inhibitor.
63
What is varicella?
A highly contagious viral infection caused by the varicella zoster virus, characterized by a blistering rash and acute fever, occurring mainly in children but can also affect adults ## Footnote Disseminated varicella occurs in immunocompromised individuals and adults and carries high morbidity
64
Who is primarily affected by disseminated primary varicella?
Immunocompromised individuals and adults ## Footnote Carries high morbidity.
65
What characterizes herpes zoster?
A painful, blistering rash caused by the reactivation of the varicella zoster virus ## Footnote More common in adults than children.
66
What is defined as cutaneous dissemination in herpes zoster?
More than 20 vesicles outside the area of the primary and adjacent dermatomes.
67
What is Sweet syndrome?
- An autoinflammatory condition - Often linked with systemic disease - Characterized by: - fever - painful inflamed or blistered skin and - mucosal lesions.
68
What are common characteristics of Sweet syndrome lesions?
Appear on the **neck and limbs** as: - papules - vesicles - plaques - erosions, or - ulcers.
69
What is dermatitis herpetiformis linked to?
Coeliac disease ## Footnote A rare but persistent immunobullous disease.
70
What age group and demographic does dermatitis herpetiformis predominantly affect?
Caucasians aged 15–40, more common in males.
71
What are the characteristics of lesions in dermatitis herpetiformis?
Extremely itchy papules and vesicles on normal or erythematous skin.
72
What is the most likely diagnosis for Ben, who has an itchy, blistering rash and a family history of celiac disease?
Dermatitis herpetiformis.
73
What type of biopsy should be taken for dermatitis herpetiformis?
A skin biopsy from a fresh blister for hematoxylin and eosin staining and a biopsy from perilesional skin for direct immunofluorescence.
74
What does hematoxylin and eosin staining reveal in dermatitis herpetiformis?
Neutrophilic microabscesses in the dermal papillae, with possible subepidermal blisters.
75
What does direct immunofluorescence show in dermatitis herpetiformis?
Granular IgA deposits in the dermal papillae.
76
What blood test can be taken for dermatitis herpetiformis?
Presence of anti-tissue transglutaminase antibodies.
77
List some complications of coeliac disease.
* Aphthous ulcers and angular cheilitis * Dental problems such as thin enamel * Dry skin and nail and hair abnormalities * Neurological problems such as ataxia, polyneuropathy, and epilepsy * Heart problems such as pericarditis and cardiomyopathy * Recurrent miscarriages * Fatty liver * Non-Hodgkin lymphoma.
78
How long does it take to notice a difference in skin symptoms on a gluten-free diet?
Several months.
79
What is the initial dose of dapsone for dermatitis herpetiformis?
25–50 mg daily.
80
How quickly does itch respond to dapsone therapy?
Within 48–72 hours.
81
What should be monitored before initiating dapsone therapy?
Routine blood tests including full blood count, urea and electrolytes, liver function tests, and glucose-6-phosphate (G6DP) deficiency.
82
What alternative can be used if there is an allergy or intolerance to dapsone?
Sulfapyridine.
83
What are some potential side effects of dapsone?
* Neurologic: Headache, dizziness, peripheral neuropathy * Cutaneous: Morbilliform eruption, urticaria, Stevens-Johnson syndrome * Hypersensitivity: Hepatitis, fever, fatigue * Hematologic: Hemolytic anemia, leukopenia * GI: Anorexia, nausea * Renal: Nephrotic syndrome.
84
What is the prognosis for dermatitis herpetiformis?
Usually good, with the majority responding well to a strict gluten-free diet and medication.
85
How often should Ben have blood monitoring throughout treatment?
Monthly for the first 3 months.
86
When can dapsone be gradually weaned off?
In those who have been on a stable gluten-free diet.
87
What are the two types of herpes simplex virus?
HSV-1 (affects the face) and HSV-2 (affects the genitals) ## Footnote HSV can be transmitted to other areas of the body.
88
What sensation may individuals experience before the rash appears in herpes simplex infection?
Tingling, burning, or itching sensation ## Footnote This occurs in the affected area prior to the development of clusters of vesicles.
89
Where does the herpes simplex virus remain after the initial infection?
In the nerve supply, remaining latent indefinitely ## Footnote This latency can lead to recurrences.
90
What causes recurrences of herpes simplex virus?
Proliferation of the virus within the epidermis of the affected dermatome ## Footnote This leads to the reappearance of symptoms.
91
What is the primary viral infection caused by the varicella-zoster virus?
Primary varicella infection (chickenpox) ## Footnote Characterized by the development of papules and vesicles.
92
How does varicella spread in individuals?
The spread pattern can be localized or disseminated ## Footnote This varies from person to person.
93
In which age group does varicella generally occur?
In children ## Footnote Adults can also be affected, usually experiencing more severe symptoms.
94
What prodromal symptoms do most adults experience before a varicella rash?
Fever, malaise, and headache ## Footnote These symptoms occur prior to the rash development.
95
What is herpes zoster also known as?
Shingles ## Footnote It is caused by the reactivation of the varicella-zoster virus.
96
What systemic symptoms may patients present with before the rash in herpes zoster?
Malaise, fever, and abnormal skin sensation ## Footnote These symptoms occur a few days prior to rash development.
97
How is herpes zoster characterized?
Localized, blistering, painful rash usually affecting 1 dermatome ## Footnote In disseminated cases, several dermatomes may be affected.
98
Does herpes zoster typically cross the midline?
Typically, it does not cross the midline ## Footnote However, it can in some cases.
99
What is the typical distribution of vesicles in herpes zoster?
Confined to 1–2 adjacent dermatomes and unilateral ## Footnote Presentation usually cuts off at the anterior and posterior midlines.
100
What may occur in immunocompromised individuals with herpes zoster?
Multidermatomal or disseminated herpes zoster ## Footnote These cases require careful monitoring.
101
What type of investigations may be required for atypical or disseminated herpes zoster?
Further investigations ## Footnote This is necessary to distinguish from other vesicular dermatoses.
102
What tests are used for diagnosing herpes zoster?
Viral swabs, bacterial swabs, and blood tests ## Footnote Blood tests may include a full blood count, liver function tests, and others.
103
What general measures should be taken for varicella zoster management?
Keep the rash clean and dry, apply paraffin gel, avoid adhesive dressings, and cover burst vesicles with non-adherent dressings. ## Footnote Non-adherent dressings such as Mepitel can be used for burst areas.
104
Which oral antivirals are recommended for varicella zoster?
Aciclovir, valaciclovir, and famciclovir. ## Footnote These should be started within 72 hours of rash onset.
105
When should oral antiviral therapy be considered for herpes zoster?
* Immunocompromised individuals (non-severe cases) * Non-truncal involvement * Moderate to severe rash or pain * Individuals over 50 ## Footnote Treatment is also warranted after 72 hours if new lesions appear or complications arise.
106
What is the role of corticosteroids in herpes zoster treatment?
May be considered in the first 2 weeks of rash onset in immunocompetent adults to reduce pain and promote healing. ## Footnote Should only be used in combination with antivirals.
107
What types of analgesics can be used for herpes zoster pain?
* Over-the-counter analgesics like paracetamol +/- codeine * Nonsteroidal anti-inflammatory drugs like ibuprofen * Strong opioids like morphine for severe pain ## Footnote Specialist advice should be sought for severe pain management.
108
What is herpes zoster ophthalmicus?
A complication of herpes zoster affecting the ophthalmic division of the trigeminal nerve. ## Footnote Complications can include keratitis, corneal ulceration, and potentially blindness.
109
What characterizes herpes zoster oticus?
Lesions in the ear, facial paralysis, and hearing/vestibular problems. ## Footnote Also known as Ramsay Hunt syndrome.
110
Define post-herpetic neuralgia.
Pain persisting for or appearing more than 90 days after initial rash onset. ## Footnote It is common in individuals over 50 and may include symptoms like burning sensation and increased sensitivity.
111
What are potential skin complications from varicella zoster?
Superinfection and secondary bacterial infections, potentially leading to cellulitis or sepsis. ## Footnote Usually involves staphylococcal or streptococcal bacteria.
112
What severe complications can arise from disseminated varicella zoster?
Pneumonia, encephalitis, and hepatitis. ## Footnote These complications can be life-threatening and are more likely in severely immunocompromised individuals.
113
What is pemphigus foliaceus?
An autoimmune disorder characterized by fluid-filled bullae on the scalp, face, and trunk. ## Footnote Most commonly appears in individuals aged 50–60, with little to no mucous membrane involvement and no systemic symptoms.
114
What are the characteristics of lesions in pemphigus foliaceus?
Fluid-filled bullae that are often scaly and crusty on an erythematous base. ## Footnote Lesions primarily occur on the scalp, face, and trunk.
115
True or False: Pemphigus foliaceus usually causes systemic symptoms.
False ## Footnote Pemphigus foliaceus does not usually cause systemic symptoms.
116
What is bullous impetigo?
A bacterial skin infection characterized by thin-roofed bullae that easily rupture. ## Footnote Lesions can develop on various body regions and can spread due to autoinoculation.
117
Where do lesions typically develop in bullous impetigo?
On the trunk, buttocks, face, extremities, and perineal regions. ## Footnote These lesions can rupture, leaving a collarette of scale.
118
What systemic symptoms may bullous impetigo cause?
Fever, malaise, and lymphadenopathy. ## Footnote Unlike pemphigus foliaceus, bullous impetigo may cause systemic symptoms.
119
What causes staphylococcal scalded skin syndrome (SSSS)?
Release of endotoxins from toxigenic strains of Staphylococcus aureus. ## Footnote SSSS primarily occurs in children under 5 and older immunocompromised individuals.
120
What are the initial symptoms of SSSS?
Systemic symptoms such as fever and widespread erythema of the skin. ## Footnote Within 24–48 hours, small fluid-filled blisters form.
121
What happens to the skin in SSSS?
The top layer of skin begins to peel, resembling a burn or scald. ## Footnote The rash can spread to other areas of the body.
122
How is bullous impetigo commonly diagnosed?
By clinical presentation and bacterial swab of a deroofed blister. ## Footnote Confirmation of S. aureus can be achieved through swabbing.
123
What is non-bullous impetigo?
A highly contagious bacterial infection caused by S. aureus or Streptococcus pyogenes. ## Footnote It typically starts as a single erythematous macule that develops into a pustule or vesicle.
124
What is impetiginization?
Secondary infection of wounds or skin conditions with S. aureus or S. pyogenes. ## Footnote This leads to the development of impetigo.
125
What is the difference between bullous and non-bullous impetigo?
Bullous impetigo is nearly always caused by S. aureus and can enter intact skin; non-bullous typically affects damaged skin. ## Footnote Bullous impetigo is more likely to cause systemic symptoms.
126
What toxins do S. aureus produce in bullous impetigo?
Exfoliative toxins, exfoliatins A and B. ## Footnote These toxins target intracellular adhesion molecules, leading to blister formation.