Dermatology Secondary Care Flashcards
(94 cards)
What is bullous pemphigoid?
A chronic, autoimmune, subepidermal blistering skin disorder.
How does bullous pemphigoid differ from pemphigus?
Pemphigus - intra-epidermal blistering, painful not pruritic
Pemphigoid - sub epidermal blistering, painful and pruritic
What is the median age of onset in bullous pemphigoid?
80 years
What is the autoantigen in bullous pemphigoid and what is its function?
Type XVII collagen (COL17)
in the basement membrane
What are three risk factors for bullous pemphigoid?
Lichen planus/psoriasis
NSAIDS/furosemide/captoptil
Radiotherapy
How does bullous pemphigoid present?
Urticarial/erythematous rash on limbs precedes blisters - urticarial prodrome
Blisters/bullae occur in skin flexures, are fluid filled
Post inflammatory hyperpigmentation/milia
Patient is otherwise well
How is bullous pemphigoid diagnosed?
Skin biopsy followed by direct immunofluorescence (DIF)
Deposit of IgG and C3 on the epidermis side of the dermal/epidermal junction
What is seen on the histology of a bullous pemphigoid patient?
Subepidermal blister with fibrin and inflammatory infiltrate and eosinophilic predominance. Intact epidermis
What is the management of bullous pemphigoid?
Urgent referral to secondary care/admission
Dermovate cream
If more severe, PO prednisolone
If systemic steroid treatment lasts more than one month, what is given?
PPI and bisphosphonates
What is a complication of bullous pemphigoid?
Secondary infection and sepsis
What is pemphigus?
A group of autoimmune disorders where there is blistering of the skin and/or mucosal surfaces.
What is the pathophysiology of pemphigus?
Circulating IgG autoantibodies bind to antigens (desmosomes) on the surface of keratinocytes leading to acantholysis (keratinocytes separate from each other)
What are some risk factors for pemphigus?
Female
Older age
Asian and Ashkenazi Jews
Pregnancy and stress
What are the main three subtypes of pemphigus?
Pemphigus vulgaris
Pemphigus foliaceus
Paraneoplastic pemphigus
How does the skin symptoms of pemphigus present?
Blisters on normal or erythematous skin and are flaccid
Affected skin is painful but rarely pruritic
Intertriginous areas - granulation and crusting
What are the other symptoms of pemphigus?
Nail changes
Burst gingival, buccal, and palatine bullae that are painful and slow healing.
Conjunctivae, oesophagus, and genitalia
How is pemphigus definitively diagnosed?
Skin biopsy from the edge of a blister, histology and DIF/IDIF
ELISAs for DSG1 and DSG3 in serum
What are the three phases of management of pemphigus?
Control, consolidation, maintenance.
What are the treatment options in the control phase of pemphigus management?
PO prednisolone
2nd line: plasmaphresis 3xw w/ azathioprine or cyclophosphamide
3rd line: IVIG
Describe the consolidation and maintenance phases of management of pemphigus.
Step down regime and tapering after 80% lesions have healed
Steroid sparing agents sometimes used to further reduce dose
What are the complications of pemphigus?
Secondary infection
Effects of corticosteroids
Malignancy immunosuppression
Give four differences between pemphigus and bullous pemphigoid
Pemphigus: younger, flaccid epidermal bullae, not pruritic
Pemphigoid: Older, tense subepidermal bullae, pruritic
What is lichen planus?
A pruritic, popular eruption characterized by its violaceous colour and polygonal shape.