Current management of generalized pustular psoriasis

Kodali, Nilesh; Blanchard, Isabella; Kunamneni, Sruthi; Lebwohl, Mark

doi:10.1111/exd.14765

Cited by 32 publications

(19 citation statements)

References 122 publications

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“…[3][4][5][6] The clinical course of GPP is heterogeneous, with patients experiencing relapsing disease with recurrent flares or persistent disease with intermittent flares. 2,6 If left untreated, GPP may become life-threatening. 7 Estimating the global prevalence of GPP is challenging because of the heterogeneity of the condition; however, a recent review of epidemiology estimated that between 1.76 and 124 patients per million of the general population are affected by GPP, depending on their geographic region.…”

Section: Introductionmentioning

confidence: 99%

Regional differences in the prevalence of generalized pustular psoriasis in Japan

Fujita,

Iwasaki,

Tsuboi

et al. 2024

The Journal of Dermatology

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Generalized pustular psoriasis (GPP), a rare form of psoriasis, is characterized by neutrophil‐rich, sterile pustules. In Japan, GPP has intractable and rare disease designation, which allows patients to access support from national and local governments for medical expenses. Previously, similar numbers of patients in Tokyo and Hokkaido have been shown to have GPP designation, despite different population sizes. Here, we determine whether there are regional differences in the proportion of patients receiving GPP designation status in Japan and aim to identify causal factors. In this descriptive, retrospective cohort study, publicly available data were collected on the number of patients with intractable and rare disease designation for GPP in each prefecture and age classification (April 2018–March 2021). Three other designated intractable and rare disease cohorts were included: pemphigus, rare skin diseases, and all diseases. The primary outcome was the standardized morbidity ratio (SMR) of patients at prefecture level (observed numbers divided by expected). Regional differences were compared with the statistical expectation for the total population and age distribution of each prefecture. Regional differences were observed in all cohorts. Overall, 1910 patients had GPP as a designated intractable and rare disease in 2020. Regional differences in SMRs for GPP were observed with high SMRs (≥1.5) in Hokkaido, Tottori, Kagawa, and Miyazaki, and low SMRs (<0.6) in Gunma and Kanagawa. Regional differences in SMRs for GPP did not correlate with the number of medical doctors or dermatologists or internal migration. The number of medical doctors or dermatologists correlated with SMRs in the rare skin diseases and total cohorts. Regional differences in Japan exist in the number of patients with GPP who have an intractable and rare disease designation. Managing rare diseases is an important public health issue, and further research is required to elucidate the factors contributing to these differences.

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Section: Introductionmentioning

confidence: 99%

Regional differences in the prevalence of generalized pustular psoriasis in Japan

Fujita,

Iwasaki,

Tsuboi

et al. 2024

The Journal of Dermatology

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“…1 Psoriasis is an immune-mediated dermatosis characterized by dysregulated keratinocyte proliferation which presents as scattered erythematous plaques with superimposed scale. 2 Psoriasis is believed to be a risk factor for type 2 diabetes mellitus (T2DM), and 11.6% of psoriasis patients have comorbid T2DM. 3 Inflammation and chronic immune stimulation common to both diseases may contribute to diabetes-associated complications in patients with comorbid psoriasis.…”

Section: Introductionmentioning

confidence: 99%

The Influence of Psoriasis on Type 2 Diabetes Mellitus Patient Profiles: A National Inpatient Sample Study

Kodali,

Blanchard,

Kumar

et al. 2023

Journal of Psoriasis and Psoriatic Arthritis

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Background Psoriasis is believed to be a common comorbidity of type 2 diabetes mellitus (T2DM). Little is known on the impact psoriasis has on T2DM patients’ disease profiles. Objective To assess the impact psoriasis has on T2DM patients’ demographics, comorbidities, and health care outcomes. Methods We retrospectively analyzed the 2017 U.S. National Inpatient Sample (NIS) database. We utilized ICD-10 codes to determine T2DM and psoriasis patients along with associated comorbidities. Continuous variables were compared by independent-sample t-tests and categorical variables were compared via Pearson chi-square. All analysis were conducted in IBM SPSS 25. Results Among 7,705,988 T2DM admissions, 0.67% of them had comorbid psoriasis. T2DM psoriasis patients (64.38; SD: 12.403) were, on average, younger (64.38 vs 66.73; P < .001) and white (78.7% vs 63.1%; P < .001) and had increased foot ulcers (4.2% vs 3.8%; P < .001), hyperglycemia (22.4% vs 21.0%; P < .001), retinopathy (22.4% vs 21.0%; P < .001), hypercoagulopathy (8.5% vs 6.9%; P < .001), and hypertension (72.5% vs 70.4%; P < .001) than T2DM patients without psoriasis. T2DM psoriasis patients spent more days in the hospital (5.49 vs 5.37; P < .001), had more concurrent diagnoses (19.05 vs 16.5; P < .001), less total charges ($60,596.71 vs $61,534.66; P = 0.010) and had less in-hospital deaths (2.0% vs 2.7%; P < .001) than T2DM patients without psoriasis. Conclusions The presence of comorbid psoriasis significantly impacts T2DM patients’ demographics, comorbidities, and health care outcomes. These findings underscore the importance of early disease monitoring, cross-specialty collaboration, and medication monitoring in order to guide individualized management strategies and optimize patient care.

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“…Due to mutations in the NF1 gene, it largely affects the development and growth of peripheral nerve cells, leading to the formation of neurofibromas, which are non‐cancerous neoplasms. Because of the aberrant development of such masses all over the body, NF1 can engender a wide range of manifestations, including skin abnormalities, bone deformities, vision problems and cardiovascular issues 2–4 . As such presentations highly vary among patients, NF1 is often difficult to manage, as there is no one‐size‐fits‐all approach to treatment 5 .…”

Section: Introductionmentioning

confidence: 99%

“…Because of the aberrant development of such masses all over the body, NF1 can engender a wide range of manifestations, including skin abnormalities, bone deformities, vision problems and cardiovascular issues. [2][3][4] As such presentations highly vary among patients, NF1 is often difficult to manage, as there is no one-size-fits-all approach to treatment. 5 However, by elucidating associations between symptoms in NF1, treatment can be better customized for patients.…”

Section: Introductionmentioning

confidence: 99%

The role of scoliosis on the comorbidity and demographics of neurofibromatosis type 1 patients: A retrospective analysis of the National Inpatient Sample database

Kodali,

Kumar,

Schwartz

2023

Experimental Dermatology

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Neurofibromatosis type 1 (NF1) is the most common neurocutaneous syndrome in the United States, affecting every 1 in 3000 individuals. NF1 occurs due to non‐functional mutations in the NF1 gene, which expresses neurofibromin, a protein involved in tumour suppression. As a result, NF1 typically presents with non‐cancerous neoplasm masses called neurofibromas across the body. Out of all NF1 abnormalities, the most common skeletal abnormality seen in around 10%–30% of NF1 patients is scoliosis, an improver curvature of the spine. However, there is a lack of research on the effects of scoliosis on demographics and morbidities of NF1 patients. We performed a national analysis to investigate the complex relationship between NF1 and scoliosis on patients' demographics and comorbidities. We conducted a retrospective cross‐sectional analysis of the 2017 US National Inpatient Sample database using univariable Chi‐square analysis and multivariable binary logistic regression analysis to determine the interplay of NF1 and scoliosis on patients' demographics and comorbidities. Our query resulted in 4635 total NF1 patients, of which 475 (10.25%) had scoliosis and 4160 (89.75%) did not. Demographic analysis showed that NF1 patients with scoliosis were typically younger, female and white compared to NF1 patients without scoliosis. Comorbidity analysis showed that NF1 patients with scoliosis were more likely to develop malignant brain neoplasms, epilepsy, hydrocephalus, pigmentation disorders, hypothyroidism, diabetes with chronic complications and coagulopathy disorders. NF1 patients with scoliosis were less likely to develop congestive heart failure, pulmonary circulation disease, peripheral vascular disease, paralysis, chronic pulmonary disease, lymphoma and psychosis. NF1 patients with scoliosis were predominantly younger, female, white patients. The presence of scoliosis in NF1 patients increases the risks for certain brain neoplasms and disorders but serves a protective effect against some pulmonary and cardiac complications.

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Current management of generalized pustular psoriasis

Cited by 32 publications

References 122 publications

Regional differences in the prevalence of generalized pustular psoriasis in Japan

Regional differences in the prevalence of generalized pustular psoriasis in Japan

The Influence of Psoriasis on Type 2 Diabetes Mellitus Patient Profiles: A National Inpatient Sample Study

The role of scoliosis on the comorbidity and demographics of neurofibromatosis type 1 patients: A retrospective analysis of the National Inpatient Sample database

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