Parotitis coexisting with Kawasaki disease in a child: a rare presentation of Kawasaki disease

Case report

A five-year-old girl presented with a five-day history of fever, earache, left side parotid swelling and a three-day history of vomiting and generalised rash. She had been treated with amoxycillin clavulanate for suspected serous otitis media. Physical examination at admission revealed bilateral cervical lymphadenopathy ≤1.5 cm, tonsillary enlargement, an erythematous rash on the body and a painful erythematous, warm, and non-fluctuant left parotid. Her lips were dry and also erythematous. No strawberry tongue or mouth ulcers were noticed. Laboratory investigations at admission showed a white blood cell count of 19,300/mm³ with 86% neutrophils, a platelet count of 305/mm³, an erythrocyte sedimentation rate of 58 mm/h, a C-reactive protein level of 10.1 mg/dL (normal range <0.5), a serum amylase of 1728 U/L (normal range = 28–100 U/L), lipase 151 U/L (normal range < 60 U/L), alanine aminotransferase 218 U/L (normal range = 18–63 U/L), aspartate aminotransferase 92 U/L (normal range = 10–32 U/L) and an albumin level just at the lower level of normal. Serologic tests for adenoviruses, mumps, enteroviruses, cytomegalovirus and Epstein–Barr virus were negative. Soft-tissue neck ultrasonography revealed multiple lymph nodes and a heterogeneous parenchyma of parotid gland. On the seventh day of her illness, she developed bilateral non-exudative conjunctivitis with erythematous oedema of palms and soles; on the ninth day with persistent fever, desquamation was observed on her body. Laboratory tests were unchanged apart from a significant drop in amylase levels to 214 U/L. KD was suspected owing to the child’s continuing fever, cervical lymphadenopathy, conjunctivitis, polymorphous rash and desquamation. An echocardiography was performed and this demonstrated diffuse dilation of the left main coronary artery measuring 3.73 mm (Figure 1). Treatment with intravenous immunoglobulin (IVIG) at a dose of 2 g/kg and oral aspirin 75 mg/kg/day was commenced. Her fever subsided 48 h later. Laboratory parameters returned to normal six days later. Aspirin doses were decreased to anti-aggregant levels. A check echocardiogram then showed only mild ectasia of the left coronary artery. Informed consent was obtained from the patient’s parents. OPEN IN VIEWER

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Table 1.

Demographic data, clinical/laboratory features and treatment of child patients with KD associated with parotitis in the literature.

	Case 1*	Case 2 †	Case 3 ‡	Case 4 §	Present case
Demographic data
Age at diagnosis (months)	60	20	3	13	60
Gender	Female	Female	Male	Male	Female
Duration of fever from onset of disease (days)	5	1	3	3	5
Duration of rash from onset of disease (days)	2	No rash on admission	No rash on admission	No rash on admission	3
Duration of facial swelling from onset of disease (days)	U	1	3	3	5
Clinical features
Fever	+	+	+	+	+
Looked ill and irritable	+	+	+	U	+
Cervical lymphadenopathy	–	+	–	–	+
Day of diffuse maculopapular rash	+	7th	–	–	+
Day of oral mucosa changes	+	+	5th	–	+
Day of erythema of palms and soles	–	–	–	–	7th
Day of oedema of hands or feet	+	12th	5th	–	7th
Day of illness that periungual desquamation occurred	5th	12th	U	U	9th
Day of bilateral bulbar conjunctival injection	+	4th	–	5th	7th
Enlargement of the parotid gland	+	12th	+	+	+
Tenderness of the parotid gland	+	–	–	+	+
Hyperaemia of the parotid gland	–	+	–	+	+
Tenderness in the parotid gland	+	+	+	+	+
Fluctuation of the parotid gland	–	–	+	–	–
Laboratory examinations
Leucocyte (/mm 3 )	13.7	U	266,000	282,000	305,000
Platelets count on admission (/mm 3 )	397	U	11,820	28,200	19,300
Erythrocyte sedimentation rate (mm/h)	57	68	U	U	92
C-reactive protein (mg/dL)	1.8	4	U	U	58
Aspartate aminotransferase (U/L)	209	U	8.7	7.73	10.1
Alanine aminotransferase (U/L)	305	U	U	U	218
Gamma glutamyl transferase (U/L)	U	U	U	U	199
Serum salivary gland amylase on admission (U/L)	354	101	46	362	1728
Peak level of serum amylase (U/L)	509	U	U	U	1728
Peak level of serum lipase (U/L)	U	U	U	U	151
Leucocytes in the urine on admission	+	+	U	U	+
Radiological investigations
Ultrasound scan	U	U	U	U	Showed parotitis
Tomography	–	Parotid enlargement Left cervical LAP	Increased intensity in the left parotid gland and the adjacent lymph nodes were enlarged	Increased intensity in the right parotid gland, and the adjacent lymph nodes were enlarged	–
Echocardiography
	Normal	Normal	Diffuse dilatation of the left main coronary artery measuring 2.8 mm and saccular dilatation of the proximal left anterior descending artery measuring 4.0 mm	Normal	Diffuse dilatation of the left main coronary artery measuring 3.73 mm
Serologic investigations	Negative	Negative	Mumps IgM positive	Negative	Negative
The day of diagnosis	5th	12th	5th	5th	9th
Initial therapy	U	Antibiotics	Antibiotics	Antibiotics	Supportive treatment
Therapy	U	IVIG and aspirin	IVIG + aspirin	IVIG + aspirin	IVIG + aspirin
Follow–up thrombocytosis	+	+	U	U	+
Day of the illness occurred thrombocytosis	12th	12th	U	U	14th
Recurrence	–	–	U	–	–

*

Seyedabadi et al.

†

Douvoyiannis et al.

‡

Do et al.

§

Yokoyama et al.

U, unknown; IVIG, intravenous immunoglobulin.

Discussion

KD is a systemic vasculitis affecting small and medium-sized vessels and is most often seen in children aged 6 months–5 years. Its aetiology is still unknown but clinical, laboratory and epidemiological features suggest that one or more transmissible agents could be the triggering factor for the inflammatory response. ¹ Typical KD criteria include fever necessarily lasting >5 days, cervical adenopathy, non-purulent bilateral conjunctivitis, oral mucosal ulceration, hand and foot oedema, and a polymorphic skin. Four of five clinical criteria are required for a definite diagnosis.

The fact that no viral agents were identified by serologic tests and fever persisted with significant features of the disease made us suspect that the parotitis was an uncommon initial presentation of KD. This has only been described in four children in the literature to date (Table 1).^2–5 Involvement of the parotitis in KD might not be due to viral or bacterial infection, but rather be due to KD itself.