Flashcards in Bullous disorders Deck (18):
Name a few causes of intraepidermal blistering?
Name 2 causes of subepidermal blistering
What does bullous pemphigoid look like?
Large tense bullae on normal skin or an erythematous base.
Most cases are in people aged >60yrs.
When the bullae burst in pemphigoid, what is left behind?
Erosions (not scarring)
How does the pre-bullous phase present in bullous pemphigoid?
Itchy erythematous plaques and papules may be the presenting feature.
What is Nikolsky's sign?
Your health care provider may use a pencil eraser to test for Nikolsky sign. The eraser is placed on your skin and gently twirled back and forth. If the test result is positive, a blister will form in the area, usually within minutes. A positive result is usually a sign of a blistering skin condition.
Bullous pemphigoid is Nikolsky sign NEGATIVE.
What does immunofluorescence show in Bullous pemphigoid?
linear IgG + complement deposited around the BM
Treatment of localised and generalised bullous pemphigoid?
Localised - Topical steroid
Generalised - oral steroid (prednisolone 40-80mg per day)
Prognosis of bullous pemphigoid?
Chronic and self limiting
Most patients achieve remission in 3 - 6 months.
Pemphigus vulgaris (turn over)
Incidence ranges from <1 – 5 new cases per million per year
Usually a disease of middle age
Flaccid vesicles/bullae on scalp, face, axillae, groins
Lesions rupture – raw, denuded erosions
Nikolsky sign POSITIVE
Oral lesions (80%) (painful erosions)
Erosions of vulva, conjunctivae, pharynx, larynx, oesophagus, rectum
How does pemphigus vulgaris present on immunofluorescence?
- "chicken wire" pattern
- IgG antibodies are directed against intercellular adhesions.
- Common to all variants of pemphigus is the process of acantholysis = lysis of intercellular adhesion sites
Prognosis of pemphigus vulgaris?
Most cases remit within 3 - 6 years.
Mortality rate 10-20% even in patients who are treated.
Who does dermatitis herpetiformis affect?
Affects all ages, majority of patients are young adults.
Dermatitis herpetiformis associations?
There may be a family history of DH or a personal history of Coeliac disease
What sites are involved in dermatitis herpetiformis?
- extensor aspects elbows and forearms
- buttocks and scapulae
- extensor aspects of knees
- face and scalp
Clinical features of dermatitis herpertiformis?
- Small blisters on an erythematous urticarial base.
- Itch usually precedes blistering.
- Grouping of lesions.
- Mucous membrane involvement is rare!!
Immunofluorescent appearance of dermatitis herpetiformis?
sub-epidermal bullae with granular IgA in dermal papillae