ABSTRACT
El uleritema ofriógenes es un trastorno cutáneo benigno y poco frecuente que se presenta habitualmente en la infancia. Se caracteriza por pápulas foliculares eritematosas y queratósicas en el lateral de las cejas, que con el tiempo suelen evolucionar a alopecia cicatricial. Dicha entidad puede aparecer como manifestación clínica aislada o asociada a varios síndromes congénitos (18p-, Cornelia de Lange, Noonan y Rubinstein- Taybi, entre otros). Presentamos el caso de un paciente de 13 años con síndrome 18p- que consultó por lesiones puntiformes rugosas al tacto y pérdida de pelo en ambas cejas (uleritema ofriógenes), así como por hiperqueratosis pilar en brazos. Esta tríada, conocida como síndrome de Zouboulis, ha sido poco descrita en la literatura. Se considera que el reconocimiento del uleritema ofriógenes es de crucial importancia ya que, ante su presencia, debería realizarse una anamnesis y una exploración física exhaustivas en búsqueda de otras alteraciones que pudieran orientar a la existencia de un trastorno genético subyacente.
Ulerythema ophryogenes is a benign and rare skin disorder commonly presenting in childhood. It is characterized by erythematous and keratotic follicular papules located on the side of the eyebrows, and which over time tends to evolve into scarred alopecia. This entity may appear as an isolated clinical manifestation or associated with several congenital syndromes (18p-, Cornelia de Lange, Noonan, Rubinstein-Taybi, among others). We present a 13-year-old male with 18p- syndrome who consults for rough lesions and hair loss in both eyebrows (ulerythema ophryogenes), as well as for hyperkeatosis pilaris in both arms. This triad, known as Zouboulis syndrome, has been rarely reported in the literature. We consider that the recognition of ulerythema ophryogenes is of crucial importance since, in view of its presence, comprehensive anamnesis and physical examination should be performed in search of other alterations that could guide the existence of an underlying genetic disord
Subject(s)
Humans , Male , Adolescent , Chromosome Disorders , Darier Disease , Abnormalities, Multiple , Chromosomes, Human, Pair 18 , Chromosome Deletion , Eyebrows/abnormalitiesABSTRACT
No abstract available.
ABSTRACT
A case of ulerythema ophryogenes (UO) is reported.A 12-year-old boy presented with erythema and follicular papules on the eyebrows and cheeks for 7 years.The lesions started as follicular papules surrounded by erythema,then spread symmetrically to the cheeks and forehead followed by the loss of eyebrows.There was no complaint of pruritus.Physical examination showed pinhead- to grain-sized,smooth,slightly indurated follicular hyperkeratotic papules surrounded by erythematous halo on the eyebrows,forehead and cheeks.Both eyebrows were nearly completely lost.Histological analysis of lesions from eyebrows revealed dilated follicular infundibulum with orthokeratotic plugs,sparse perivascular and perifollicular lymphohistiocytic infiltrate,widened and sclerotic collagen fibers in the dermis.According to the clinical manifestations and histopathological findings,the patient was diagnosed with ulerythema ophryogenes,and given oral vitamin A 2.5 million unit once a day,vitamin E 100 mg once a day,topical vitamine E cream twice a day,0.025％tretinoin ointment once at night.Two weeks later,the lesions improved.