[Harlequin phenomenon associated with neurological abnormalities: A case report]. / Phénomène d'Arlequin associé à des anomalies neurologiques : un cas.
Ann Dermatol Venereol
; 143(5): 369-71, 2016 May.
Article
en Fr
| MEDLINE
| ID: mdl-27021901
BACKGROUND: Harlequin phenomenon consists of facial flush and erythrosis with unilateral sweating and pallor, associated with contralateral anhidrosis. We present the case of a child in whom the syndrome was associated with Horner's syndrome, epilepsy, mental and psychomotor retardation. PATIENTS AND METHODS: A 9-year-old boy presented with right unilateral hemifacial erythema on effort, with normal colouring and Horner's syndrome on the left side of the face. His medical history revealed generalized myoclonic epilepsy, psychomotor delay and mental retardation. No underlying anomalies were identified. Harlequin phenomenon was diagnosed. DISCUSSION: Despite its stereotypical clinical features, Harlequin phenomenon is a poorly known disease. However, clinicians must be aware of it in order to determine the diagnosis and investigate for causes and any associated abnormalities. The underlying mechanism is an autonomic neuropathy affecting the sympathetic vasodilator neurons. To our knowledge, there have been no previous reports of Harlequin phenomenon in association with Horner syndrome, psychomotor delay and mental retardation.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Enfermedades del Sistema Nervioso Autónomo
/
Síndrome de Horner
/
Rubor
/
Hipohidrosis
Tipo de estudio:
Prognostic_studies
/
Risk_factors_studies
Límite:
Child
/
Humans
/
Male
Idioma:
Fr
Revista:
Ann Dermatol Venereol
Año:
2016
Tipo del documento:
Article
Pais de publicación:
Francia