Management of Pustular Psoriasis

Treatment Approach

The British National Formulary recommends moderately potent topical corticosteroids (grade III) for symptom relief in localized pustular psoriasis, with topical coal tar and dithranol providing some benefit 1
Initial management of generalized pustular psoriasis usually requires hospital admission and systemic therapy, with treatment options determined by disease extent and severity 1, 2
The National Health Service recommends avoiding systemic corticosteroids in pustular psoriasis due to the risk of disease exacerbation upon discontinuation, but short-term therapy may be considered during severe systemic phases 3

Acitretin is contraindicated in pregnancy or women planning pregnancy within 2 years of stopping treatment, with a recommended dose of 0.1-1 mg/kg/day 3
Cyclosporine is contraindicated in abnormal renal function, uncontrolled hypertension, and previous/concomitant malignancy, with a recommended initial dose of 2.5 mg/kg/day taken twice daily 3
Methotrexate is contraindicated in pregnancy, breastfeeding, significant hepatic damage, and blood disorders, with a recommended dose for acute generalized pustular psoriasis and psoriatic erythroderma 3, 4

PUVA (psoralens plus ultraviolet A) is generally considered the systemic treatment of first choice with the least toxicity, with an efficacy rate of 45.7% reported in generalized pustular psoriasis, and is contraindicated in pregnancy, significant cataracts, age <18, and previous cutaneous malignancy 3
Ultraviolet B radiation may be helpful but must be used with caution as it may exacerbate pustular psoriasis 1

Combination therapy should be approached with extreme caution as toxicity is at least additive, with acitretin combined with narrowband UVB showing synergistic effects 3
All systemic treatments require appropriate pretreatment assessment and ongoing monitoring, with regular blood tests, liver function tests, and serum creatinine monitoring essential depending on the agent used 3

Systemic corticosteroids can precipitate erythrodermic psoriasis, generalized pustular psoriasis, or very unstable psoriasis when discontinued 5, 6
The British Medical Journal recommends avoiding oral steroids in pustular psoriasis due to the risk of disease exacerbation 5, 6

Systemic corticosteroids should be restricted to rare and specific conditions, including persistent uncontrollable erythroderma causing metabolic complications, generalized pustular psoriasis of the von Zumbusch type, and hyperacute psoriatic polyarthritis that threatens severe irreversible joint damage 5, 6

Acitretin is particularly effective for pustular psoriasis, with response seen as early as 3 weeks (dosed at 0.1 to 1 mg/kg/day), according to the Journal of the American Academy of Dermatology 7
Biologics, such as infliximab and etanercept, have shown rapid efficacy in severe generalized pustular psoriasis, as reported in the British Journal of Dermatology 8
The American Academy of Dermatology recommends acitretin combined with narrowband UVB (NB-UVB) for synergistic effects in pustular psoriasis 7

If a patient is experiencing a flare during tapering of oral steroids, the British Medical Journal recommends not abruptly discontinuing steroids, as this may worsen the flare 5, 6
The Journal of the American Academy of Dermatology suggests initiating acitretin therapy (0.1-1 mg/kg/day) while slowly tapering the steroids 7
For severe cases, the British Journal of Dermatology recommends considering adding a biologic agent such as infliximab 8

Infliximab has shown rapid efficacy in severe generalized pustular psoriasis, with complete disease clearance in some cases, as reported by the British Journal of Dermatology 9, 10, 11
The British Journal of Dermatology recommends infliximab for severe generalized pustular psoriasis, with generally positive results, including complete disease clearance 9, 10, 11

Etanercept at 50 mg biweekly has shown clinical efficacy in a case series of 6 patients with generalized pustular psoriasis, with maintenance of response for up to 48 weeks, as reported by the British Journal of Dermatology 11

TNF antagonists should be avoided in chronic palmoplantar pustulosis, as they may exacerbate the condition, according to the British Journal of Dermatology 9, 10, 12, 13
The British Journal of Dermatology suggests that TNF antagonists, such as etanercept, infliximab, and adalimumab, are reasonable to recommend for acropustulosis if it has a major impact on quality of life 9, 10, 12, 13

The American Academy of Dermatology provides a Grade C recommendation for infliximab in pustular psoriasis, indicating it "may be recommended" for other subtypes of moderate-to-severe psoriasis 14, 15, 16, 17

Infliximab demonstrates rapid and often complete disease clearance in severe generalized pustular psoriasis, with the British Association of Dermatologists reporting generally positive results, including complete disease clearance in documented cases 18, 19
The British Association of Dermatologists reports that infliximab has been used with generally positive results, including complete disease clearance allowing withdrawal of all conventional systemic treatments in documented cases 18, 19
Two of three patients in a follow-up study achieved complete clearance with infliximab treatment 18, 20, 19

Standard dosing is 5 mg/kg infused at weeks 0, 2, and 6, then every 8 weeks thereafter, as recommended by the American Academy of Dermatology 14, 15, 16
Dose intensification up to 10 mg/kg and/or more frequent intervals (as often as every 4 weeks) may be needed for better disease control, with a Grade B recommendation from the American Academy of Dermatology 14, 15, 16, 17

Infliximab may be combined with methotrexate to augment efficacy, with a Grade B recommendation from the American Academy of Dermatology, which appears particularly beneficial for long-term management of generalized pustular psoriasis 14, 15, 16
Combination with topical high-potency corticosteroids with or without vitamin D analogues can be recommended, with a Grade B recommendation from the American Academy of Dermatology 14, 15, 16

TNF antagonists including infliximab should be avoided in chronic palmoplantar pustulosis, as they may exacerbate this condition, according to the British Journal of Dermatology 19
Upon discontinuation of infliximab, relapse is common but not universal, with the British Association of Dermatologists case series showing two of three patients relapsed after stopping infliximab following variable numbers of infusions 18, 20, 19

Etanercept at 50 mg biweekly has also demonstrated efficacy in generalized pustular psoriasis, with maintenance of response up to 48 weeks, according to the British Journal of Dermatology 18, 20, 19

The National Rheumatology Society recommends IL-17 inhibitors, IL-12/23 inhibitors, and IL-23 inhibitors for psoriatic disease, including skin manifestations, with strong evidence for psoriatic skin disease in the context of psoriatic arthritis 21
The American College of Rheumatology suggests that selection of biologic agents should be influenced by head-to-head study results, comorbidities, and activity in other disease domains 21

The European Academy of Dermatology and Venereology recommends oral PUVA combined with acitretin as a superior treatment option compared to either alone for generalized pustular psoriasis 22