Insulin dependent diabetes and goitre in a case of hypomelanosis of Ito (Incontinentia Pigmenti Achromians).

Incontinentia Pigmenti Achromians (IPA) or, more aptly, Hypomelanosis of Ito is now largely accepted as a Neurocutaneous Disorder. Studies, so far, have focussed on the ultrastructural abnormalities and to our knowledge, there have been no attempts to ascertain if there is a particular HLA pattern. We describe an interesting case where IPA occurred in association with Insulin Dependent Diabetes as well as a goitre, suggesting that HLA studies may well be worthwhile. The literature is also reviewed.