Cutaneous and Extracutaneous Manifestations of Behçet’s Disease Linked to Its Disease Activity and Prognosis

Figures & data

Figure 1 Recruitment process and the study flow diagram.

Table 1 Demographic and Clinical Data of Patients with Behçet’s Disease

Characteristics	(N = 119)
Demographic data
Mean age at diagnosis (years) ± SD	35.7 ± 10.7
Sex; N (%)
Male	48 (40.3%)
Female	71 (59.7%)
Median disease duration from first diagnosis of BD (months) (IQR)	96 (48–168)
Median BDCAF score (IQR)	5 (5–7)
Median cumulative dose of corticosteroids (milligrams of prednisolone) (IQR)	9856.25 (3125–18,350)
Morbidity; proportion (%)	42/91 (46.2%)
Median hospital admission duration (days) (IQR)	7 (5–11.5)
Mortality; proportion (%)	1/60 (1.7%)

Abbreviations: BD, Behçet’s disease; BDCAF, Behçet’s disease current activity form; IQR, interquartile range; SD, standard deviation.

Table 2 Frequency of Cutaneous and Extracutaneous Manifestations of the Patients with Behçet’s Disease

Characteristics	(N = 119)
Cutaneous manifestations; N (%)
Recurrent oral ulcers	116 (97.5%)
Recurrent genital ulcers	71 (59.7%)
Erythema nodosum	42 (35.3%)
Papulopustular lesions	29 (24.4%)
Cutaneous vasculitis	9 (7.6%)
Other cutaneous lesions (Sweet’s syndrome-like lesions, PG-like lesions, and EM-like lesions)	6 (5.0%)
Positive pathergy test result; proportion (%)	18/39 (46.2%)
Extracutaneous manifestations; N (%)
Ocular manifestations	72 (60.5%)
Articular manifestations	64 (53.8%)
Neurological manifestations	11 (9.2%)
Gastroenterological manifestations	9 (7.6%)
Vascular manifestations	5 (4.2%)
Cardiac manifestations	0 (0%)

Abbreviations: EM, erythema multiforme; PG, pyoderma gangrenosum.

Figure 2 A recurrent oral ulcer on the buccal mucosa.

Figure 3 Recurrent oral ulcers at the uvula and tonsil.

Figure 4 A recurrent genital ulcer at the vulva.

Figure 5 A recurrent genital ulcer on the scrotum.

Figure 6 Erythema nodosum-like lesions on the lower extremities.

Table 3 Cutaneous Manifestations in Relations to Behçet’s Disease Activity

Clinical Manifestations	Median BDCAF score without Clinical Manifestations (IQR)	Median BDCAF Score with Clinical Manifestations (IQR)	Univariate Quantile Regression Analysis	Multivariate Quantile Regression Analysis
			p-value	p-value
Minor oral ulcer at the buccal mucosa	7 (5–7)	5 (5–7)	<0.001	0.003
Minor oral ulcer at the tonsil	5 (5–7)	7.5 (7–8)	0.045	0.024
Papulopustular lesions at the neck	5 (5–7)	8 (7–8)	<0.001	1.000
Papulopustular lesions at the lower extremities	7 (5–8)	5 (5–5)	0.023	1.000
Erythema nodosum	5 (5–7)	7 (5–7)	<0.001	0.123

Abbreviations: BDCAF, Behçet’s disease current activity form; IQR, interquartile range.

Table 4 Clinical Factors in Relations to Cumulative Dose of Corticosteroids

Clinical Factors	Cumulative Dose of Steroids without Clinical Factor (milligrams) (Median, IQR)	Cumulative Dose of Steroids with Clinical Factor (milligrams) (Median, IQR)	Multivariable Analysis (Adjusted for Follow-Up Time)
			p-value
Gender	Male 14,610 (6235–23,645)	Female 6732 (1810–14,290)	0.003
Recurrent genital ulcers	14,405 (5812.5–23,645)	7202.5 (1152.5–14,210)	0.010
Ocular manifestations	4903.75 (420–14,270)	14,210 (6322.5–23,366.25)	0.002

Abbreviation: IQR, interquartile range.

Table 5 Clinical Factors in Relations to Behçet’s Disease Morbidity

Clinical Factors	Univariate Logistic Regression Analysis			Multivariate Logistic Regression Analysis
	OR	95% CI	p-value	OR	95% CI	p-value
Gender
Male	3.33	[1.40, 7.96]	0.007	2.78	[1.10, 6.98]	0.031
Female	0.30	[0.13, 0.72]	0.007	0.35	[0.13, 0.91]	0.031
Ocular manifestations
Present	5.22	[2.01, 13.54]	0.001	4.39	[1.57, 12.29]	0.005
Absent	0.19	[0.07, 0.50]	0.001	0.23	[0.08, 0.64]	0.005

Abbreviations: CI, confidence interval; OR, odds ratio.