DERM Revision 3 Flashcards
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?
In frail patients
Autoimmune blistering condition
Antibodies against basement membrane proteins which are key components of hemidesmosomes
* BP 180
* BP 230
Which neurological conditions is Bullous Pemphigoid associated with? [3]
Which medications are Bullous Pemphigoid associated with? [2]
Neurological disease (that cause frailty)
* Stroke
* Dementia
* Parkinson’s Disease
Drugs:
* PD-1 Inhibitors (used in the treatment of metastatic melanoma)
* Gliptins (diabetic medication)
Describe the clinical manifestations of Bullous Pemphigoid [5]
- 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
Describe the topical treatments [4] and systemic therapy [5] used to treat Bullous Pemphigoid
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
Describe the pathophysiology of Pemphigus Vulgaris [1]
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
Describe the clinical features of pemphigus vulgaris [+]
- 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)
Which drugs can induce PV? [2]
Penicillamine
ACE-inhibitors
Dx of Pemphigus Vulgaris ?
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
Tx of Pemphigus Vulgaris? [4]
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
Tx for Dermatitis Herpetiformis? [2]
Gluten free diet for life
Dapsone
Define erythroderma [1]
Name 5 causes
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
Tx for Eczema Herpeticum? [2]
Acyclovir
Flucoxacillin
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
Describe in detail the skin lesions seen in SJS / TENs [4]
- Erythematous or purpuric macules
- Diffuse erythema
- Targetoid/ atypical target lesions
- Flaccid blisters
What is a classic differentiating feature between pemphigoid and pemphigus? [2]
Bullous pemphigoid:
- mouth is usually spared
- blisters are more tense
Pemphigus vulgaris:
- Mouth commonly involved
- blisters commonly burst
Complications associated with BP? [4]
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
PV can be a pareneoplastic disease of which cancers? [2]
Lymphomas
Castleman disease
Dapsone can be used to treat dermatitis herpetiformis.
Name some side effects of using as treatment [2]
hemolytic anaemia and methemoglobinemia
- regular monitoring of complete blood counts and methemoglobin levels.
Patients with dermatitis herpetiformis have an increased risk of developing [], a rare but aggressive form of non-Hodgkin lymphoma.
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.
Explain some complications of erythroderma
Secondary infection
Acute renal failure
- water loss from skin; immune deposits in kidney
High output cardiac failure
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
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