A Case of Incomplete and Atypical Kawasaki Disease Presenting with Retropharyngeal Involvement.

Background: Kawasaki disease (KD) is a childhood acute febrile vasculitis of unknown aetiology. The diagnosis is based on clinical criteria, including unilateral cervical lymphadenopathy, which is the only presenting symptom associated with fever in 12% of cases. A prompt differential diagnosis distinguishing KD from infective lymphadenitis is therefore necessary to avoid incorrect and delayed diagnosis and the risk of cardiovascular sequelae. Case presentation: We describe the case of a 4 years old boy presenting with febrile right cervical lymphadenopathy, in which the unresponsiveness to broad-spectrum antibiotics, the following onset of other characteristic clinical features and the evidence on the magnetic resonance imaging (MRI) of retropharyngeal inflammation led to the diagnosis of incomplete and atypical KD. On day 8 of hospitalisation (i.e., 13 days after the onset of symptoms), one dose of intravenous immunoglobulins (IVIG; 2 g/kg) was administered with rapid defervescence, and acetylsalicylic acid (4 mg/kg/day) was started and continued at home for a total of 8 weeks. Laboratory examinations revealed a reduction in the white blood cell count and the levels of inflammatory markers, thrombocytosis, and persistently negative echocardiography. Clinically, we observed a gradual reduction of the right-side neck swelling. Fifteen days after discharge, the MRI of the neck showed a regression of the laterocervical lymphadenopathy and a resolution of the infiltration of the parapharyngeal and retropharyngeal spaces. Conclusion: Head and neck manifestations can be early presentations of KD, which is frequently misdiagnosed as suppurative lymphadenitis or retropharyngeal infection. A growing awareness of the several possible presentations of KD is therefore necessary. Computed tomography (CT) or MRI can be utilised to facilitate the diagnosis.

Complex Investigation of a Pediatric Haematological Case: Haemophagocytic Syndrome Associated with Visceral Leishmaniasis and Epstein⁻Barr (EBV) Co-Infection.

BACKGROUND: Visceral leishmaniasis (VL) is an anthropozoonosis caused by an intracellular parasite belonging to the genus Leishmania. In the Mediterranean region, L. donovani and L. infantum are responsible for VL and dogs are the main reservoir. Haemophagocytic lymphohistiocytosis (HLH) represents a complication of VL and consists of unrestrained activation and proliferation of lymphocytes and macrophages, leading to uncontrolled immune activation. Haemophagocytic lymphohistiocytosis may also develop during viral infection, and Epstein⁻Barr virus (EBV) infection is one of the main HLH causes. Macrophage haemophagocytosis in the bone marrow aspirate is pathognomonic. CASE PRESENTATION: The case involves a 19-month-old male infant presenting with a high persistent fever with a fluctuating pattern, pancytopaenia, hepatosplenomegaly, and a high triglyceride level. Initial investigations showed an EBV infection. Considering the persistent signs and symptoms, bone marrow aspiration was performed and confirmed the suspicion of HLH. In addition, the presence of Leishmania infection was shown. The patient was treated with liposomal amphotericin B and had complete resolution of his symptoms. CONCLUSION: Diagnosis of VL represents a demanding challenge in endemic and non-endemic areas. Our case demonstrates that leishmaniasis should always be considered in the differential diagnosis in patients presenting with hepatosplenomegaly and cytopaenia with a persistent fever, even in cases of infectious mononucleosis. Moreover, the execution of bone marrow aspiration should not be delayed in order to diagnose and treat at an early stage the potential occurrence of VL, especially if complicated with HLH.

Early Desquamating Perineal Erythema in a Febrile Infant: A Characteristic Clinical Feature of Kawasaki Disease.

Background: The occurrence of a distinctive perineal eruption that appears early in infants with Kawasaki disease (KD), the most relevant type of febrile vasculitis of childhood, has received little attention in pediatric reports. KD diagnosis is based on clinical criteria, which can be supported by laboratory abnormalities or positive echocardiography findings: difficulty in diagnosis can be increased by incomplete or atypical presentations, but a timely diagnostic process is essential in the youngest patients who are more prone to the risk of cardiac sequelae resulting from KD. Case Presentation: In this report, we present the case of a 2-month-old infant with an unusual presentation of KD, in whom diagnosis was made despite fever remission on the fourth day of hospitalization following intravenous corticosteroid therapy to treat concomitant bronchoconstriction. The presence of early desquamating perineal erythema led to the consideration of KD diagnosis, confirmed by the echocardiographic assessment of right and left coronary artery dilatations with pericardial effusion on the fifth day of hospital stay. Conclusions: Diagnosis of KD represents a demanding challenge, mainly when the illness starts with an incomplete or nuanced presentation. An erythematous desquamating perineal rash is a valuable early clinical clue, which might facilitate a prompt diagnosis of KD. This case emphasizes that an accurate assessment of all clinical features, including perineal erythema with early tendency to desquamation, and an eventual echocardiography, are necessary in an infant displaying fever to corroborate the suspicion of KD.