Primary Raynaud's phenomenon in an infant: a case report and review of literature

Raynaud's phenomenon (RP) was first described by Maurice Raynaud in 1862 [1]. Classically, the initial description of RP involved triphasic color changes in the digits, with blanching (white) leading to cyanosis (blue) followed by reactive hyperemia (red) [2,3]. However, it has been realized that not every patient experiences all 3 phases of color change and the majority of patients present with uniphasic color change involving an isolated bluish discoloration of digits commonly known as acrocyanosis [4-6]. Unlike RP, acrocyanosis is a common phenomenon in infants and young children [4-7]. Acrocyanosis is generally bilateral, symmetric and involves hands and feet. Since infantile acrocyanosis is a benign and self-resolving condition, it does not require medical attention [7,8]. Rarely, acrocyanosis in infants can be caused by RP and may require immediate medical attention to prevent complications of RP [9-13]. In this report, we describe an infant who initially presented with unilateral acrocyanosis and was diagnosed to have primary RP based on his subsequent clinical course. In view of the rarity of RP in infants and young children, the literature about RP is reviewed with a specific focus on the pediatric population. To help differentiate benign acrocyanosis from acrocyanosis associated with other serious conditions, the differential diagnosis of unilateral and bilateral acrocyanosis in infants is also discussed.A one-month-old healthy male infant was brought to his pediatrician's office for the evaluation of bluish to blackish discoloration of his left hand. His mother incidentally noted this color change while she was changing his diaper. She did not recall any trauma or insect bite. She denied using naphthalene balls in the storage area for the infant's clothes. This history was helpful to exclude methemoglobinemia as exposure to naphthalene balls can cause infantile acrocyanosis [7]). He was breast-fed and his mother was the primary care taker. The infant's bi