Drug Reaction with Eosinophilia and Systemic Symptoms Syndrome in a Child with Cystic Fibrosis

Table 1

Patient’s diagnosis of DRESS syndrome based on the RegiSCAR system.

Clinical parameters	Present/absent (yes/no)	Patient score	RegiSCAR system
Fever (≥38.5)	Yes	0	No/unknown = −1
			Yes = 0
Lymphadenopathy	No	0	>1 cm, two sites; yes = 1
			No/unknow = 0
Eosinophilia	Yes	2	Eosinophil count of 0.7–1.49 × 10^9 = 1
			Eosinophil count of ≥1.5 × 10^9 = 2
Atypical lymphocyte	Unknown	0	Yes = 1
			No/unknown = 0
Skin rash			Suggestive features: ≥2 facial edemas, purpura, infiltration, desquamation (no = −1; unknown = 0; yes = 1)
(i) Extent ≥50% of BSA	Yes	1
(ii) Rash suggestive of DRESS	Yes	1
Organ involvement	Yes	1	1 point for each organ involvement, maximum score: 2
Disease duration ≥15 days	No	−1	No/unknown = −1
			Yes = 0
Skin biopsy suggesting DRESS	Not applicable	0	No: −1
			Yes/unknown: 0
Exclusion of other causes	Yes	1	1 point if ≥3 of the following tests were -ve: HAV, HBV, HCV, EBV, mycoplasma, chlamydia, ANA, blood culture
Total score		5

Total score <2 = = no case. Total score 2–3 = possible case. Total score 4–5 = probable case. Total score >5 = definite case. BSA = body surface area; HAV = hepatitis A virus; HBV = hepatitis B virus; HCV = hepatitis C virus; EBV = Epstein-Barr virus; ANA = antinuclear antibodies.