Treatment of acute pustular psoriasis

If a person presents with suspected generalised pustular psoriasis or erythrodermic psoriasis this should be managed as a medical emergency. Arrange for immediate same-day specialist dermatology assessment and ongoing management. (1)

Disease-specific medications include systemic retinoids (acitretin, isotretinoin), methotrexate, cyclosporine, and infliximab (2)

• methotrexate and cyclosporin usually provide the most rapid response (1)
  • methotrexate can be used
    • as a short term treatment method
    • to control the disease before starting other methods of treatment
    • frequently as long term maintenance treatment method (1)

In the paediatric age group, acitretin, cyclosporine, methotrexate, and etanercept are considered the first line options (1).

Second line options:

• include systemic treatments like etanercept and adalimumab or topical treatments like corticosteroids, calcipotriene, and tacrolimus
  • all of these options can be monotherapy choices or in combination with the first line options
  • phototherapy is another treatment option
  • early delivery is recommended in pregnant patients with impetigo herpetiformis
  • there are reports that indicate the successful use of L-1 receptor antagonists (e.g., anakinra) and IL-36 receptor antagonists in treating pustular psoriasis
    • tocilizumab, a monoclonal antibody against the IL-6 receptor, has also shown efficacy in some recalcitrant cases of pustular psoriasis

Attention must be given to fluid balance and body temperature.

References:

1. Romiti R, Hirayama ALDS, Arnone M, et al; Generalized pustular psoriasis (von Zumbusch). An Bras Dermatol. 2022 Jan-Feb;97(1):63-74.
2. Shah M, Al Aboud DM, Crane JS, et al. Pustular Psoriasis. [Updated 2019 Jul 5]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan-.