Paget Disease, Extramammary

Extramammary Paget disease is a cancerous disease seen at various sites, most often in the anogenital region. The clinical features are usually those of eczematous eruptions with weeping and crust formation. This disease has been shown to be a skin manifestation of internal malignancy. In a study of 40 patients with Paget disease of the anogenital region, Helwig and Graham (1963) did not find a family history of the disease in any of the cases. Kuehn et al. (1973) described a case occurring in a father and son. The father, aged 66, presented with extramammary Paget disease in the right scrotal area. No mention was made of other family history.