Members of two different families were found to have the dysplastic naevus syndrome and coexistent endocrine abnormalities. The dysplastic naevus syndrome is probably inherited as an autosomal dominant trait and has been associated with other primary malignancies. This is the first time that it has been described in association with endocrine abnormalities.

A study of melanocytic naevi was carried out in southern Spain to examine the relationship between numbers of naevi at different body sites as predictors of whole-body naevus count and to determine whether the naevus count on the arms is valid for identifying the risk factors for total naevi. Subjects were the control group from a case-control study on risk factors for cutaneous melanoma. They ...

Blue rubber bleb naevus syndrome (BRBNS) is a rare clinical entity characterised by multiple haemangiomas commonly occurring in the skin and the gastrointestinal (Gl) tract. The association between these cutaneous cavernous haemangiomas and similar lesions in the U l tract was first described in 1860 and the acronym B R B N S was given by Bean in 1958 [ l J, Patients may present with massive GI...

The case of a 5-month-old black female child with a linear sebaceous naevus syndrome and multiple congenital anomalies is presented. Ocular malformations consisted of colobomatous changes of the lid and retina, dermoid of the conjunctiva, chorioretinal changes, and peripapillary atrophy of the optic nerve. Systemic findings included midline cleft of the secondary palate with involvement by the ...