Dermatologist Dr Monika Saha discusses an interesting dermatology case with the condition Pemphigus, and gives advice on treatment.
A 36 year old man presented with two month history of painful erosions and blisters in his mouth. Later blisters appeared on his trunk. Over a course of a few weeks he developed this severe clinical picture and was referred for an urgent dermatological opinion. The presence of oral involvement and absence of a history of potentially causative drugs suggested an autoimmune bullous disease.
A skin biopsy from the back showed an intraepidermal blister and characteristic positive staining with Immunoglobulin G and C3 intercellularly. These features confirmed a diagnosis of pemphigus vulgaris (PV).
- Pemphigus is a rare, autoimmune blistering disease, which can affect both the skin and mucous membranes. Patients often present with oral ulceration first, followed by erosions and superficial fragile blisters classically on the upper body
- The disease can also affect the eyes, throat, oesophagus and genitalia
- Pemphigus is due to antibodies, which target the molecular bridges between keratinocytes in the skin. The damage to these bridges leads to blister formation
- The disease can last from 1 year to more than 30 years
- All ethnic groups can be affected but is more common in the Indian, Ashkenazi Jewish and Mediterranean populations.
- Pemphigus was a life threatening disease. These days, the disease cannot be cured but can be controlled on steroids and immunosuppression
- The mainstay of treatment is a combination of systemic steroids with oral prednisolone, 0.5-1mg/kg, topical potent steroids eg Dermovate, steroid mouth-washes eg Betnesol, and an immunosuppressive agent, such as azathioprine or mycophenolate mofetil
- Patients often remain on low dose steroids (5-15mg prednisolone) and an immunosuppressive adjuvant for some years
- Patients are reviewed and monitored 3-6 monthly in secondary or tertiary care.
Other diagnoses to consider
- Bullous pemphigoid is 5 times more common than pemphigus in the UK. Bullous pemphigoid usually presents in the older patient and is associated with widespread tense blisters with a lack of oral disease
- Other autoimmune diseases include dermatitis herpetiformis, pemphigus foliaceus, epidermolysis bullosa acquisita, paraneoplastic pemphigus and mucous membrane pemphigoid.
- Non- autoimmune causes of similar blisters include drug eruptions, bullous lichen planus and infections such as disseminated HSV or staphylococcal scalded syndrome
The role of the GP
- Who to suspect? Patients with an increasing number of flaccid or tense blisters on the skin. Patients with chronic mouth ulcers, urticaria (pre-bullous pemphigoid), genital soreness and ulceration, difficulty swallowing and eye symptoms
- When to refer? We need to review as an urgent outpatient referral or as an emergency depending on severity
- Monitoring for steroid side effects of treatment is important – Blood pressure checks and glucose testing
- Most patients on steroids will be on a calcium/vitamin D supplement and those found to have evidence of osteopenia or osteoporosis or those over 65 years of age will require a bisphosphonate
- If patients flare in between outpatient appointments, steroid dose can be temporarily increased e.g. prednisolone 20-40mg and slowly weaned over a few weeks back to their baseline maintenance dose. Guidance from the dermatologist should be sought at this stage.
- Patients on immunosuppressives will initially be monitored closely by the hospital, but in the maintenance phase of treatment they will require 3 monthly blood test monitoring (FBC, U&E, LFT).
Dr Monika Saha is a Consultant Dermatologist at The London Skin and Hair Clinic.