British Association of Dermatologists' Guidelines for the Management of Pemphigus Vulgaris 2017

K.E. Harman; D. Brown; L.S. Exton; R.W. Groves; P.J. Hampton; M.F. Mohd Mustapa; J.F. Setterfield; P.D. Yesudian


The British Journal of Dermatology. 2017;177(5):1170-1201. 

In This Article

Nursing Care

PV has the potential to cause extensive cutaneous erosion, and in very active cases, fragility of normal skin (exhibited by a positive Nikolsky sign). Therefore, careful handling of the skin by specialist dermatology nurses, or other nursing staff familiar with caring for patients with skin failure, is essential. Attention to fluid balance, haemodynamic stability, thermoregulation, prevention of infection, prevention of further skin trauma, pain management, nutritional intake and psychological support is equally important in addition to skincare.[245]

It is recommended that any intact bullae are decompressed by piercing. The blister roof is left in situ to act as a biological dressing. A daily blister chart is a useful means of mapping disease progress in the acute phase.[246]

A Guide to Blister Management

Anecdotal experience suggests that aspirating blisters causes more discomfort than piercing them. Table 2 summarizes the management of blisters for all types of bullous disease including PV and epidermolysis bullosa.

The application of a bland emollient, such as 50% white soft paraffin and 50% liquid paraffin, is recommended to support barrier function,[247] reduce transcutaneous water loss and encourage re-epithelialization.[248,249] This should be applied to the whole skin including erosions. Products containing irritants and sensitizers should be avoided. To reduce the shearing forces and pain associated with application of emollients to erosions, a 50 : 50 aerosolized preparation of white soft paraffin:liquid paraffin can be used to supplement application of the ointment form. Emollients can be applied directly to the skin or initially to primary dressings.

Potassium permanganate soaks (one Permatab® – 400 mg – in 4 L of water, i.e. a 1 : 10 000 solution) may be helpful for wet, weepy erosions. The solution should not be applied for longer than 15 min as it becomes ineffective due to oxidation. If practical, soaking in a bath is an effective way of treating large areas. Alternatively, it can be applied by soaking gauze swabs or dressing pads and applying to affected areas. The patient should be counselled regarding temporary skin discoloration. Nails should be covered with white/yellow soft paraffin to help prevent nail discoloration.[250–252]

There is no clear evidence regarding the superiority of any particular dressing in PV, but those used should be nonadherent.[253] The application of an emollient and dressing to eroded areas helps reduce fluid and protein loss, reduces the risk of secondary infection and assists with pain control. A soft silicone mesh dressing, such as Mepitel®, is a suitable primary dressing, and it can be coated (spread) with an appropriate emollient such as a 50 : 50 mix of liquid paraffin and white soft paraffin, or a topical antimicrobial if appropriate, prior to application to the skin. The secondary dressing usually needs to be absorbent, such as a soft silicone foam or other foam dressing, for example Mepilex® or Allevyn®.[253] These dressings can be secured to the trunk or the limbs with soft knitted tube dressings such as Comfifast®.

When dressings are removed, if they have dried onto the skin, they should be soaked off to minimize pain and avoid further damage.[254] There is no evidence regarding the optimal frequency of dressing changes, but one should consider the appearance of strikethrough on the secondary dressing, the need to assess for evidence of infection and the stage of wound healing. In the acute stage, dressings should be changed daily to assess them. It may be appropriate in the later stages of healing to change only the secondary dressing but leave the primary dressing in situ, with the underlying erosion left undisturbed. Further applications of topical agents can be placed on top of the silicon mesh primary dressing in this situation. Crusts should be removed to promote healing. All patients with pemphigus should be nursed on an appropriate pressure-relieving mattress regardless of the degree of skin failure as they are prone to developing pressure areas by virtue of the disease.[255]

Infection and sepsis are a significant risk and a major cause of mortality in PV, so vigilance in detecting signs of infection is essential.[256] Infection also increases the risk of scarring. Daily washing with an antibacterial product can decrease colonization. Dressings should be changed using an aseptic technique and patients with extensive erosions barrier nursed. Erosions showing clinical signs of infection should have bacterial and viral swabs sent. It may be appropriate to apply topical antimicrobials for short periods only. Systemic antibiotics should be used if there are local or systemic signs of infection or extending infection of the skin. Local policy should guide the choice of antibiotic agent.

Pain control is essential, and attention needs to be paid to both acute and maintenance (background) analgesia with the ability to provide timely additional short-term boosts when needed, for example with dressing changes. The advice of a pain team may be necessary.