blistering disorders

Question 1

what is bulbous pemphigoid

Answer 1

Bullous pemphigoid is an autoimmune subepidermal blistering disease

secondary to the development of antibodies against hemidesmosomal proteins BP180 and BP230

Question 2

Who gets bulbous pemphigoid?

Answer 2

common in elderly patients

more prevalent in older patients with neurological disease, particularly stroke, dementia and Parkinson disease.

precipitated by phototherapy

Question 3

what drugs are associated with bulbous pemphigoid

Answer 3

PD1-inhibitor immunotherapies (such as pembrolizumab, nivolumab) used to treat metastatic melanoma and other cancers.

gliptins

penicillamine
potassium iodide
frisemide
captopril
gold
penicillin
sulfasalazine
topical fluorouracil.

Question 4

clinical features of bullous pemphigoid

Answer 4

• itch
• (usually) large tense bullae (fluid-filled blisters)
• rupture forming crusted erosions.
• precipitated by a nonspecific rash for several weeks before blisters appear

the blisters usually heal without scarring

• localised to one area, or widespread on the trunk and proximal limbs.

flexural

Blisters inside the mouth and in genital sites are uncommon.

Question 5

complications of bulllous pemphigoid

Answer 5

• Bacterial staphylococcal and streptococcal skin infection and sepsis
• Viral infection with herpes simplex, varicella or herpes zoster
• Complications of treatment
• Underlying and associated diseases.

Question 6

Ix for bullous pemphigoid

Answer 6

immunofluorescence shows IgG and C3 at the dermoepidermal junction

Question 7

management for bullous pemphigoid

Answer 7

• wound dressing where required, monitor signs of infection
• topical steriods for localised disease
• oral corticosteroids combination
  of oral tetracycline and nicotinamide, immunosuppressive agents (e.g. azathioprine, mycophenolate mofetil, methotrexate, and other)

Question 8

what is pemphigus vulgaris

Answer 8

A blistering skin disorder which usually affects the middle-aged

autoimmune disease that is characterised

• painful blisters
• erosions on the skin
• mucous membranes, most commonly inside the mouth

Question 9

causes of pemphigus vulgaris

Answer 9

Autoantibodies against antigens within the epidermis causing an intra-epidermal split in the skin

Question 10

clinical features of pemphigus

Answer 10

• lesions on the mucous membranes such as the mouth and genitals.
• thin-walled flaccid blisters filled with clear fluid that easily rupture causing itchy and painful erosions

● Lesions are often painful
● Usually affects the mucosal areas (can precede skin involvement)

Question 11

complications of pemphigus vulgaris

Answer 11

• Secondary bacterial infection
• Fungal infection, especially candida
• Viral infection, especially herpes simplex
• Nutritional deficiencies due to difficulty eating
• Complications of systemic steroids especially infections and osteoporosis
• Complications of immune suppressive treatments
• The psychological effects of severe skin disease and its treatment (anxiety and depression)

Question 12

how is pemphigus vulgaris diagnosed?

Answer 12

a biopsy from the skin adjacent to a lesion.

Histology typically shows rounded-up and separated keratinocytes (acantholytic cells) just above the basal layer of the epidermis. Suprabasal clefting may be reported

direct immunofluorescence staining of perilesional skin biops

Question 13

treatment of pemphigus vulgaris

Answer 13

systemic corticosteroids - high doses of oral predinisolone

General measures – wound dressings where required, monitor for
signs of infection, good oral care (if oral mucosa is involved)

● Oral therapies – high-dose oral steroids, immunosuppressive
agents (e.g. methotrexate, azathioprine, cyclophosphamide,
mycophenolate mofetil, and other

Question 14

causes of bullous pemphigoid

Answer 14

● Autoantibodies against antigens between the epidermis and dermis causing a sub-epidermal split in the skin