Clinical course and disease burden of patients with generalized pustular psoriasis in Portugal: a multicenter retrospective cohort study

Figures & data

Table 1. Demographic characteristics of the sample (n = 59).

Characteristic	Value
Age at GPP diagnosis (y), mean ± SD	55.1 ± 21.3
Age at initial visit (y), mean ± SD	58.0 ± 30.8
Female, n (%)	39 (66.1)
Race
White, n (%)	50 (84.7)
Black, n (%)	2 (3.4)
Asian, n (%)	1 (1.7)
Other or unreported, n (%)	6 (10.2)
BMI (kg/m²), mean ± SD	28.0 ± 5.0
Prior History
GPP, n (%)	9 (15.3)
Plaque psoriasis, n (%)	42 (71.2)
- Age at presentation (y), mean ± SD	34.0 ± 18.7
Psoriatic Arthritis, n (%)	9 (15.3)
- Age at presentation (y), mean ± SD	36.7 ± 11.1
Family history
Pustular Pso, n (%)	0 (0)
Family history, Plaque Pso, n (%)	19 (32.2)

BMI: body mass index. GPP: generalized pustular psoriasis. Mo: months. Pso: psoriasis. SD: standard deviation. Y: years.

Table 2. Comorbidities and lifestyle habits reported in the initial visit (n = 59).

Comorbidity	n (%)
Hypertension	30 (50.8)
Obesity	21 (35.6)
Dyslipidemia	20 (33.9)
Diabetes	15 (25.4)
IBD	2 (3.4)
Liver disease	2 (3.4)
Latent tuberculosis	4 (6.8)
Hypothyroidism	1 (1.7)
Stroke	1 (1.7)
Cholangitis	1 (1.7)
Cancer	5 (8.5)
Smoking history
Never	38 (64.4)
Current	12 (20.3)
Former	5 (8.5)
Unreported	4 (10.3)
Alcohol use
None	43 (72.9)
Mild	10 (16.9)
Moderate	1 (1.7)
High	2 (3.4)
Unreported	3 (5.1)

IBD: inflammatory bowel disease.

Figure 1. Potential precipitating factors for generalized pustular psoriasis (n = 31).

Table 3. Systemic treatment option during the study period, duration of treatment cycle (months), reported adverse events and treatment discontinuation.

Treatment	N	Cycles	Duration	AE	% Discontinuation	Discontinuation
Systemic Steroids	12	12	6 (1–108)	1	66.7	Lack of Efficacy: 3
						Adverse Events: 1
						Risk of toxicity: 4
Retinoids	31	31	8 (1–36)	6	48.4	Lack of Efficacy:13
						Adverse Events:1
						Risk of toxicity:1
Cyclosporine	19	19	4 (1–120)	4	78.9	Lack of Efficacy:5
						Adverse Events:3
						Risk of toxicity:7
Methotrexate	13	13	10 (1–120)	2	69.2	Lack of Efficacy:5
						Adverse Events:2
						Risk of toxicity:2
Phototherapy	3	3	4 (2–4)	0	66.7	Lack of Efficacy:0
						Adverse Events:0
						Risk of toxicity:2
Dapsone	2	2	2, NA	1	50.0	Lack of Efficacy:1
						Adverse Events:0
						Risk of toxicity:0
Biologics
Etanercept	3	3	8 (6–108)	1	33.3	Lack of Efficacy:1
						Adverse Events:2
						Risk of toxicity:0
Adalimumab	10	10	22 (7–48)	0	30.0	Lack of Efficacy:3
						Adverse Events:0
						Risk of toxicity:0
Infliximab	2	2	7.5 (6–9)	1	50	Lack of Efficacy:1
						Adverse Events:1
						Risk of toxicity:0
Ustekinumab	3	3	43 (36–50)	1	NA	NA
Secukinumab	4	4	11 (6–24)	0	75.0	Lack of Efficacy:3
						Adverse Events:0
						Risk of toxicity:0
Ixekizumab	3	3	10 (9–32)	0	33.3	Lack of Efficacy:1
						Adverse Events:0
						Risk of toxicity:0
Risankizumab	1	1	NA	0	NA	NA
Guselkumab	3	3	18 (18–24)	0	NA	NA
Tildrakizumab	1	1	2	0	NA	NA

Duration of treatment is presented in months as median (range). A: information not available. AE: adverse events.

Table 4. Healthcare utilization during the study period in patients with generalized pustular psoriasis (n = 59).

Healthcare Facility	n (%)	Mean (SD) Annual Rate
Hospitalizations		0.33 ± 0.44
0	19 (32.2)
1	28 (47.5)
2	7 (11.9)
3	2 (3.4)
4	2 (3.4)
5	1 (1.8)
Emergency department visits		0.54 ± 0.52
0	6 (10.2)
1-2	39 (66.1)
3-4	10 (16.9)
>4	4 (6.8)
Outpatient visits		1.49 ± 1.50
0	2 (3.4)
1-2	12 (20.3)
3-4	30 (50.8)
>4	15 (25.4)