Harlequin and Horner syndromes with iris heterochromia in pediatrics: report of a congenital case

Marcella Caldeira-Camisasca-Souza, Division of Pediatric Dermatology, Fundação de Medicina Tropical Doutor Heitor Vieira Dourado - FMT HVD, Manaus, AM, Brazil Alessandra E. de Morais, Division of Pediatric Dermatology, Fundação de Medicina Tropical Doutor Heitor Vieira Dourado - FMT HVD, Manaus, AM, Brazil Naum M. Ribeiro, Departamento de Medicina, Universidade Federal do Amazonas (UFAM), Manaus, Brazil Cláudio do C. Chaves-Filho, Department of Clinical Surgery, Faculty of Medicine, Federal University of Amazonas, Manaus, Brazil Virginia V. Figueiras, Department of Dermatology, Fundação de Medicina Tropical Doutor Heitor Vieira Dourado, FMT HVD, Manaus, AM, Brazil Luciana M. Dos Santos, Department of Pediatric Dermatology, Fundação de Medicina Tropical Doutor Heitor Vieira Dourado - FMT HVD, Manaus, AM, Brazil

Harlequin syndrome is an uncommon disorder characterized by asymmetric sweating and flushing of the face and neck in response to sympathetic stimuli. This report describes a case of congenital origin associated with Horner syndrome and iris heterochromia emphasizing the rarity of this association. Male, 11 years old, presenting since birth with unilateral facial flushing when exposed to sympathetic stimuli. Physical examination revealed miosis, ptosis, enophthalmos, and iris heterochromia. After investigation, the diagnosis of Harlequin syndrome associated with Horner syndrome was confirmed. Harlequin syndrome is an autonomic disorder manifested by anhidrosis and pallor on one side of the face, with contralateral flushing and sweating in response to sympathetic stimuli. The congenital form is commonly related to other syndromes, and in such cases, treatment is conservative, with botulinum toxin considered a minimally invasive option.

Keywords: Harlequin syndrome. Horner syndrome. Iris heterochromia; congenital.