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Generalized Pustular Psoriasis (GPP)

Name of disease:

Generalized Pustular Psoriasis (GPP)

ICD-10 diagnosis code:

L40.1

Causes:

GPP is caused by a dysregulation of the IL-36 pathway, leading to excessive inflammation and the development of sterile pustules.

Pattern of inheritance:

N/A

Prevalence:

The prevalence is wide ranging from 7.46 per 1 million in Japan to 100 per 1 million in the US. GPP occurs in more women than men

Diagnosis:

GPP is diagnosed based on the presence of visible pustules on erythematous skin with confirmation from histopathology, and supportive evidence from laboratory and genetic evaluations.

Age of onset:

Often starts in people in their 40s, but it can occasionally occur at a younger age.

Common signs and symptoms:

Flares appear suddenly in the form of painful, pus-filled blisters (pustules) that may be accompanied by other symptoms, such as fever, joint pain, and extreme tiredness. GPP can also lead to other systemic complications.

Available treatments (medicinal and non-medicinal):

There is no approved treatment for GPP. Current treatment options lack the efficacy required to adequately manage GPP flares. Even with treatment, symptoms of GPP flares can persist.

Disease management tips:

N/A

References:

1. Marrakchi S, Guigue P, Renshaw BR, et al. N Engl J Med. 2011;365(77):620-628. 2. Gabay C, Towne JE. J Leukoc Biol. 2015;97(4):645-652. 3. Kharawala S, Golembesky AK, Bohn RL, et al. Expert Rev Clin Immunol. 2020;16(3):239-252. 4. Ohkawara A, Yasuda H, Kobayashi H, et al. Acta Derm Venereol (Stockh). 1996;76:68-71. 5. Ly K, Beck KM, Smith MP, et al. Psoriasis (Auckl). 2019;9:37-42.6. National Psoriasis Foundation. Pustular Psorasis. Available at: https://www.psoriasis.org/pustular. Accessed on March 10, 2023. 7. Strober B, Kotowsky N, Medeiros R, et al. Dermatol Ther (Heidelb). 2021;11(2):529-541.

Other useful websites:

Patient Group Hong Kong Psoriasis Patients' Associationhttps://www.psoriasis.org.hk/ International Federation of Psoriatic Disease Associations (IFPA)https://ifpa-pso.com/

Acknowledgment

This page is contributed by Boehringer Ingelheim (HK) Ltd.