title: Congenital Harlequin syndrome as an isolated phenomenon: A case report and review of the literature.
abstract: Harlequin syndrome (HS) is a rare autonomic disorder due to a hemifacial cutaneous sympathetic denervation. It is characterized by unilateral diminished sweating and flushing of the face even though after heat or prolonged exercise. It is typically acquired. Congenital cases only represent a 6% of all individuals with HS. All congenital HS cases reported so far showed a concomitant Horner syndrome, probably due to a stellate ganglion involvement. HS represents an uncommon autonomic disorder due to a hemifacial cutaneous sympathetic denervation. It is clinically characterized by a dramatic alteration in facial appearance: ipsilateral denervated pale and dry half from the other intact red and moist half.
  
  
  CONCLUSIONS
  We present, to the best of our knowledge, the first case of a patient with a congenital HS as an isolated phenomenon.
authors.affiliation: Department of Pediatrics, Hospital Universitario de Fuenlabrada, Madrid, Spain. Electronic address: arantxa_vidal_esteban@hotmail.com.
authors.forename: A
authors.initials: A
authors.lastname: Vidal Esteban
authors.affiliation: Department of Pediatrics, Hospital Universitario de Fuenlabrada, Madrid, Spain. Electronic address: daninatera@hotmail.com.
authors.forename: D
authors.initials: D
authors.lastname: Natera-de Benito
authors.affiliation: Department of Dermatology, Hospital Universitario de Fuenlabrada, Madrid, Spain. Electronic address: diego.martinez@salud.madrid.org.
authors.forename: D
authors.initials: D
authors.lastname: Martínez Sánchez
authors.affiliation: Department of Ophtalmology, Hospital Universitario de Fuenlabrada, Madrid, Spain. Electronic address: alberto.reche@salud.madrid.org.
authors.forename: A
authors.initials: A
authors.lastname: Reche Sainz
authors.affiliation: Department of Pediatrics, Hospital Universitario de Fuenlabrada, Madrid, Spain. Electronic address: mariarocio.rodriguez@salud.madrid.org.
authors.forename: M R
authors.initials: MR
authors.lastname: Rodríguez Díaz
authors.affiliation: Department of Pediatrics, Hospital Universitario de Fuenlabrada, Madrid, Spain. Electronic address: crisalfaroiznaola@gmail.com.
authors.forename: C M
authors.initials: CM
authors.lastname: Alfaro Iznaola
authors.affiliation: Department of Pediatrics, Hospital Clínico San Carlos, Madrid, Spain. Electronic address: tsantos.hflr@salud.madrid.org.
authors.forename: M T
authors.initials: MT
authors.lastname: de Santos Moreno
mesh_terms: D001342:Autonomic Nervous System Diseases / Q000151:congenital* / Q000175:diagnosis*; D005145:Face; D005483:Flushing / Q000151:congenital* / Q000175:diagnosis*; D006801:Humans; D007007:Hypohidrosis / Q000151:congenital* / Q000175:diagnosis*; D007223:Infant; D008297:Male; D035583:Rare Diseases / Q000151:congenital* / Q000175:diagnosis*
keywords: Autonomic nervous system; Autonomic neuropathy; Congenital; Facial flushing; Harlequin sign; Harlequin syndrome