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Generalized Pustular Psoriasis (GPP)

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Guidelines

Generalized pustular psoriasis (GPP) is a rare and chronic inflammatory skin disease that is characterized by the eruption of sterile pustules. It can manifest at any age, however, the median reported age at diagnosis is approximately 50 years. Flares in GPP can be triggered by factors such as infections and pregnancy, but they also occur de novo. These flares can cause several life-threatening complications.1

Guidelines for Diagnosis Including Clinical Features

The clinical criteria used to describe GPP varies. Since the first description of this disease in 1910, the clinical features of GPP have not been homogeneously defined, leading to a lack of standard international criteria. Therefore, varying diagnostic criteria are still in place.1 

ERASPEN Guidelines

The European Rare and Severe Psoriasis Expert Network (ERASPEN) published a consensus statement on the phenotypes of GPP in 2017.2 This ERASPEN consensus defined GPP as macroscopically visible, primary, sterile pustules that occur on nonacral skin, not restricted to psoriasis plaques.2 This consensus also specified that a diagnosis of GPP requires a relapse in disease or disease persistence for over 3 months. In addition, it stated that if an individual shows multiple types of pustular psoriasis, the classification should follow the principal characteristics.2

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JDA Guidelines

Japanese guidelines were developed by the Japanese Dermatological Association (JDA) and the Study Group for Rare Intractable Skin Disease in 2018 as an update to GPP guidelines published by Umezawa et al. in 2003.3,4 The 2003 guidelines established by Umezawa et al. presented the following as diagnostic criteria4:

  • Systemic symptoms, such as fever and malaise
  • Multiple, isolated septic pustules that develop on erythematous skin
  • Kogoj’s spongiform pustules that have been histopathologically confirmed
  • Laboratory findings consistent with systemic inflammation, including left shift leukocytosis, elevated erythrocyte sedimentation rate (ESR), elevated C-reactive protein (CRP) levels, hypoproteinemia, and hypocalcemia
  • Recurrence of clinical and histological findings

Read more about GPP signs and symptoms

These criteria were updated in 2018 to highlight that GPP is not only a dermatological disease, but a condition that causes systemic inflammation and, in some cases, severe complications. These guidelines, therefore, include the possible complications of GPP, such as mucosal and ocular symptoms, secondary amyloidosis, and arthritis.3 The laboratory abnormalities used to make a GPP diagnosis were also removed from the criteria, as these were not sufficiently sensitive. Laboratory testing is now recommended to evaluate the severity of the disease and its potential complications. The 2018 diagnostic criteria according to the Japanese guidelines are summarized as3:

  • Presence of systemic symptoms, such as fever and fatigue
  • Systemic or extensive flush accompanied by multiple sterile pustules
  • Neutrophilic subcorneal pustules that are histopathologically characterized by Kogoj’s spongiform pustules
  • Recurrence of clinical and histological findings

A definitive diagnosis of GPP can be made in patients that present with all the criteria listed above.3

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Guidelines for Treatment

There is a comparable lack of treatment guidelines for patients with GPP. However, unlike the somewhat recent updates to diagnostic guidelines, treatment indications from the 2012 Japanese and National Psoriasis Foundation (NPF) guidelines have not been as recently updated.1 

The NPF recommendations include the use of systemic medications and supportive care. Medications for GPP include oral retinoids such as Soriatane® (acitretin) and isotretinoin (sold as Absorica®, among others), methotrexate, cyclosporine (sold as Gengraf® and Neoral®), and Remicade® (infliximab), which are established as first-line therapies in adult patients. In addition, Soriatane, cyclosporine, methotrexate, and Enbrel® (etanercept) are considered first-line therapies in children.5

According to the NPF, second-line therapies include biologic agents such as Enbrel, Humira® (adalimumab), Stelara® (ustekinumab), and Cosentyx® (secukinumab), as well as topical agents, such as corticosteroids, Dovonex® (calcipotriene), and Protopic® (tacrolimus).5

Read more about GPP treatment

References

  1. Rivera-Díaz R, Daudén E, Carrascosa JM, de la Cueva P, Puig L. Generalized pustular psoriasis: a review on clinical characteristics, diagnosis, and treatment. Dermatol Ther (Heidelb). 2023;13(3):673-688. doi:10.1007/s13555-022-00881-0
  2. Navarini AA, Burden AD, Capon F, et al; ERASPEN Network. European consensus statement on phenotypes of pustular psoriasis. J Eur Acad Dermatol Venereol. 2017;31(11):1792-1799. doi:10.1111/jdv.14386
  3. Fujita H, Terui T, Hayama K, et al; Japanese Dermatological Association Guidelines Development Committee for the Guidelines for the Management and Treatment of Generalized Pustular Psoriasis. Japanese guidelines for the management and treatment of generalized pustular psoriasis: the new pathogenesis and treatment of GPP. J Dermatol. 2018;45(11):1235-1270. doi:10.1111/1346-8138.14523
  4. Umezawa Y, Ozawa A, Kawasima T, et al. Therapeutic guidelines for the treatment of generalized pustular psoriasis (GPP) based on a proposed classification of disease severity. Arch Dermatol Res. 2003;295(Suppl 1):S43-S54. doi:10.1007/s00403-002-0371-6
  5. Robinson A, Van Voorhees AS, Hsu S, et al. Treatment of pustular psoriasis: from the Medical Board of the National Psoriasis Foundation. J Am Acad Dermatol. 2012;67(2):279-288. doi:10.1016/j.jaad.2011.01.032

Reviewed by Hasan Avcu, MD, on 5/25/2023.

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