Periodic exophthalmos associated with the blue rubber bleb naevus syndrome: a case report

A 71-year-old woman with the blue rubber bleb naevus syndrome experienced 4 episodes of self-limiting unilateral exophthalmos. Is is suggested that a vascular malformation in the orbit is responsible for this phenomenon. A biopsy sample was taken of one of the cutaneous lesions and studied by light and transmission electron microscopy. The histological appearances are discussed in relation to possible mechanisms causing this periodic exophthalmos.