Familial hyperchylomicronemia syndrome is a monogenic autosomal recessive disorder that causes severe and refractory hypertriglyceridemia. This uncommon condition is challenging to diagnose and treat and can lead to comorbidities such as acute pancreatitis. Although treatment options are limited in the pediatric population, strict diets and treatments approved for other dyslipidemias may be implemented in familial hyperchylomicronemia syndrome, given the lack of pharmacological interventions available. We report a 14-year-old female presented to the emergency room with abdominal pain suggestive of acute pancreatitis. Biochemical analysis revealed a triglyceride value of 4260 mg/dL. Treatment for triglyceride reduction with a strict CHILD-2 triglyceride-lowering diet, insulin infusion, fibrates, and multiple plasmapheresis were initially insufficient. Primary hypertriglyceridemia was suspected, and genetic testing identified a homozygous pathogenic variant in the lipoprotein lipase gene, diagnosing familial hyperchylomicronemia syndrome. She was discharged with a maximum dose of fibrate, statin, omega-3 fatty acids, and a restrictive diet. At her 1-month and 9-month follow-ups, her triglyceride values were 756 and 495 mg/dL, respectively, without incident complications. Familial hyperchylomicronemia syndrome is an uncommon condition with limited available literature and treatment options, especially in the pediatric population. Acute pancreatitis secondary to severe hypertriglyceridemia is a condition with a high risk of mortality which requires prompt clinical suspicion and treatment.

Congenital cutaneous candidiasis (CCC) is a rare and usually benign disorder that develops within the first week of life. It predominantly presents with diffuse maculopapular and papulopustular erythematous desquamating patches. We report a preterm neonate with respiratory distress syndrome and skin diffuse maculopapular lesions at birth. Candida albicans was isolated from skin and gastrointestinal fluid culture; she underwent medical treatment with topical and systemic antifungal with clinical improvement and skin lesions resolution.

Congenital cutaneous candidiasis is a rare and usually benign disorder that develops within the first week of life. We report a preterm neonate with skin diffuse maculopapular lesions at birth. Candida Albicans was isolated from skin and gastrointestinal fluid culture. Antifungal treatment was initiated with skin lesions resolution.