Essential primary cutis verticis gyrata

Abstract

ResumoObjetivo: relatar um caso raro de uma criança portadora da forma primária essencial de Cutis verticis gyrata.Descrição: menino de 9 anos, apresenta importante hipertrofia da pele do couro cabeludo, com dobras que se assemelham aos giros cerebrais. Não apresenta retardo mental, nem alterações oftalmoló-gicas e não há relatos semelhantes na família.Comentários: o diagnóstico de Cutis verticis gyrata primária essencial foi estabelecido pela presença de redundância da pele do couro cabeludo e ausência de alterações neurológicas e oftalmológi-cas, tendo-se feito o diagnóstico diferencial com as formas secundá-rias que incluem: nevo intradérmico cerebriforme, paquidermoperiostose, acromegalia e doenças inflamatórias do couro cabeludo. Trata-se do único relato na literatura de uma criança com esta forma de Cutis verticis gyrata. AbstractObjective: to report a rare case of a child with essential primary Cutis verticis gyrata.Report: nine-year-old boy with extensive hypertrophy of scalp skin, with a cerebriform appearance. No underlying neurologic and ophthalmologic disorders were found, and no other cases were described in his family.Comments: the diagnosis of primary Cutis verticis gyrata was established by thickening of the scalp and absence of neurologic and ophthalmologic abnormalities. Differential diagnosis comprises secondary conditions such as: cerebriform intradermal nevus, pachydermoperiostosis, acromegaly, and inflammatory diseases of the scalp. This is the first report of a child with this form of Cutis verticis gyrata. A CVG pode ser classificada em duas formas: primária (essencial e não-essencial) e secundária 4 . A forma primária não-essencial responde por 0,5% dos pacientes com retardo mental; paralisia cerebral, epilepsia, catarata e cegueira podem estar presentes 1,5 . Já a forma primária essencial não está associada com alteração neurológica e oftalmológica, ocorre somente formação de dobras no couro cabeludo, que mimetizam os giros cerebrais; aparece na puberdade e ocorre exclusivamente em homens; o tipo de herança é incerta 1,4,5 . As formas secundárias de CVG são representadas mais freqüentemente por paquidermoperiostose, acromegalia e nevo cerebriforme intradérmico 1 . J Pediatr (RioEm crianças, os casos de CVG, independentemente da sua etiologia, são muito raros, e os poucos relatos devemse à forma primária não-essencial 6,7 , associação com sín-dromes genéticas 8,9 e casos familiares 10 (Tabela 1).

Primary Cutis Verticis Gyrata

“…There are no additional diseases associated with the primary essential form; however, the nonessential form is associated with many neurological, psychiatric, and ophthalmologic disorders. 4 The histopathologic findings may vary from a normal skin texture to a thickening of the connective tissue with hypertrophy of adnexal structures. 1 Secondary cutis verticis gyrata is more common than the primary form, and it occurs as a consequence of a number of local and systemic diseases that are not related to each other.…”

Cutis verticis gyrata

“…The estimated prevalence of CVG is 1 in 100 000 in males 0.026 in 100 000 in females 2. CVG can be classified in two categories: (1) primary (non-essential and essential) and (2) secondary.…”

“…CVG can be classified in two categories: (1) primary (non-essential and essential) and (2) secondary. The primary non-essential form may be associated with mental retardation,cerebral palsy, epilepsy, cataract and blindness while primary essential form is not associated with such abnormalities 2. The causes of secondary CVG include acromegaly, pachydermoperiostosis, psoriasis, chronic pemphigus, myxoedema, intradermal amyloidosis, intradermal cerebriform naevus, congenital melanocytic naevi, naevus lipomatosus, neurofibroma and rarely insulin resistance syndrome.…”

Cerebriform intradermal nevus-A rare clinical entity presenting as cutis verticis gyrata

“…The former can be either essential or non-essential depending on the presence of associations like cerebral palsy, epilepsy, seizure, mental retardation and the latter is caused by a wide variety of underlying conditions. Cerebriform intradermal nevus, a rare condition of the scalp is one such cause of cutis verticis gyrate [3,4].…”