Neonatal lupus with atypical cardiac and cutaneous manifestation

• Author(s): Morais, S ; Santos, IC ; Pereira, DF ; Mimoso, G
• Date: 2013
• Persistent ID: http://hdl.handle.net/10400.4/1656
• Origin: Repositório do Centro Hospitalar e Universitário de Coimbra
• Subject(s): Doenças do Coração; Lúpus Eritematoso Sistémico; Recém-Nascido
• Document Type: Article
• Language: English

Description

Neonatal lupus erythematosus is a rare, passively acquired autoimmune disease, caused by maternal autoantibodies. The most common manifestations are skin rash and congenital heart block. Cutaneous manifestations may be present at birth, but often develop within a few weeks after delivery. Congenital heart block may present as bradycardia in utero or during physical examination at birth. Approximately 40-60% of mothers are asymptomatic when the infants are diagnosed. We present a case of a child, born with erythematosus lesions in the face, scalp, trunk, limbs and nodules/papules on the palmar and plantar surfaces. He also had hepatosplenomegaly and thrombocytopenia. Echocardiography showed hyperechoic lesions on the anterior papilar muscle of the left ventricle and on the lateral cusp of the tricuspid valve. The mother had unexplained fever and vasculitic lesions in her hands and feet. Antinuclear antibodies, anti-SSa/Ro and anti-SSb/La were positive in the mother and child, making the diagnosis of neonatal lupus.