Generalized Pustular Psoriasis Exacerbation Linked to G-CSF Use

An individual with GPP diagnosed with intravascular large B-cell lymphoma was treated for neutropenia with G-CSF and exacerbation of the GPP.

A patient with generalized pustular psoriasis (GPP) who was diagnosed with intravascular large B-cell lymphoma received granulocyte colony-stimulating factor (G-CSF) — a cytokine—to alleviate the development of neutropenia.

The 48-year-old Japanese woman reported exacerbation of her GPP, according to a case study reported in The Journal of Dermatology.

The patient in this case had been receiving treatment for GPP, an inflammatory skin disorder in which individuals develop neutrophil-rich, noninfectious, sterile pustules, since 11 years of age. When undergoing systemic therapy for GPP, she reported no disease flare-ups.

Approximately 1 year prior to this report, the patient was diagnosed with intravascular large B-cell lymphoma, for which she received 3 courses of R-CHOP chemotherapy (rituximab, cyclophosphamide, doxorubicin, and vincristine) combined with intrathecal chemotherapy (methotrexate [MTX], cytarabine, and prednisolone). Further, she received filgrastim for the management of neutropenia, which developed during her first and second R-CHOP courses. During her third R-CHOP course, she received pegfilgrastim, which was associated with fever and erythema 3 days following treatment.

These results and our clinical case suggest that C-GSF could be an exacerbating factor for GPP.

Read more about GPP epidemiology

In addition, 2 courses of rituximab combined with high-dose MTX were administered after the 3 courses of R-CHOP chemotherapy. Pegfilgrastim was administered again on day 3 of her fourth R-CHOP course (ie, day 15). The patient was admitted to the hospital on day 18 of the R-CHOP course (ie, day 1) with pustules on her extremities, widespread erythema, and a high fever.

Because of the possibility of a bacterial infection, she received antimicrobial therapy on day 1 of hospitalization. On days 3 to 6, steroid pulse therapy was administered for her rash. On day 6, the fifth course of R-CHOP was administered.

Following treatment, the patient’s fever resolved, her rash and pustules improved, and the serologic inflammatory response diminished. Subsequently, no severe exacerbations of her rash were reported.

In patients with GPP, laboratory assessments are critical for evaluating disease severity, with the most frequently described abnormality reported to be leukocytosis. The key inflammatory response in individuals with GPP comprises activation of interleukin (IL) 36, which can act on keratinocytes via the IL-36 receptor to generate additional IL-36 expression and the production of neutrophil chemokines, including IL-8, which cause the attraction of neutrophils into the skin.

G-CSF is known to activate neutrophils, enhance the production of IL-8, and increase peripheral blood neutrophils, which accounts for the patient’s reaction to this treatment. A 2022 study among patients with GPP demonstrated that G-CSF and IL-8 were elevated in individuals with the condition. The analysis also revealed important links between serum cytokines, including G-CSF and IL-8 levels, and the severity of GPP.

“These results and our clinical case suggest that C-GSF could be an exacerbating factor for GPP,” the researchers concluded.

This article originally appeared on Rare Disease Advisor


Sakashita C, Matsushima Y, Yokota N, Kitagawa H, Habe K, Yamanaka K. Exacerbation of generalized pustular psoriasis after administration of granulocyte colony-stimulating factor in a patient with intravascular large B-cell lymphoma. J Dermatol. Published online June 13, 2023. doi:10.1111/1346-8138.16862