Paraneoplastic pemphigus

Abstract

Paraneoplastic pemphigus (PNP) is a rare severe variant of pemphigus which is caused by an underlying neoplasia. The (skin) abnormalities are caused by an immune response against multiple antigens, among which periplakin, envoplakin and alpha-2-macroglobulinlike protein 1 are most specific. Clinical signs can vary from lichenoid or bullous to crusted and hyperkeratotic skin lesions. Mucosal involvement is common. It is a multi-organ disease, in which lung involvement is an unfavourable prognostic factor. Treatment is difficult and consists of treating the underlying malignancy, immune suppressive medication and in rare cases lung transplantation.