Poland's syndrome and recessive X-linked ichthyosis in two brothers

Poland's syndrome consists of unilateral congenital absence of the pectoral is major muscle with a variable degree of ipsilateral upper limb deformity, The aetiology of Poland's syndrome is unknown, although an inherited tende ncy to develop a compromised embryonic vascular supply in the affected area s has been suggested. The majority of reported cases are sporadic, and in o nly a few instances is there a familial incidence. We describe the occurren ce of Poland's syndrome in two brothers who also had recessive X-linked ich thyosis. To the best of our knowledge this is the first report of such an a ssociation.