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Anais Brasileiros de Dermatologia

Print version ISSN 0365-0596On-line version ISSN 1806-4841


FIGUEIRAS, Daniela de Almeida et al. Oculoectodermal syndrome: twentieth described case with new manifestations. An. Bras. Dermatol. [online]. 2016, vol.91, n.5, suppl.1, pp.160-162. ISSN 0365-0596.

Oculoectodermal syndrome is a rare disease characterized by the association of aplasia cutis congenita, epibulbar dermoids, and other abnormalities. This report describes the twentieth case of the disease. We report a 4-year-old female child who presented with the classical features of the syndrome: aplasia cutis congenita and epibulbar dermoids. Our case expands the clinical spectrum of the disease to include: diffuse hyperpigmentation (some following the Blaschko´s lines); hypopigmented skin areas on the trunk; arachnoid cyst on the right fronto-parietal border; rounded left side of the hippocampus; and dermoid cyst underlying the bulb-medullary transition. Our patient also reported infantile hemangioma on the right wrist and verrucous hemangioma on the left leg, the latter not previously described in the literature.

Keywords : Astigmatism; Dermoid Cyst; Ecchymosis; Hemangioma; Hyperpigmentation; Hypopigmentation.

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