Lymphomatoid papulosis (LyP) is a self-healing eruption in the spectrum of CD30⁺ lymphoproliferative disorders. The most common lymphoproliferative disorder associated with LyP is the most common form of cutaneous T-cell lymphoma: mycosis fungoides.

We sought to describe a distinct entity on the spectrum of CD30⁺ lymphoproliferative disorders.

Seven patients presented with similar findings. Within a well-circumscribed area, the size and location of a patch of mycosis fungoides, these patients had continual eruptions of papulonodules that were histologically typical of LyP. The localized areas of involvement were treated as oligolesional mycosis fungoides and long-standing remissions occurred even after years of experiencing continuous localized eruptions. The clinical and histologic findings are reviewed and presented in a way to further the identification of patients with this entity.

This distinct entity is only defined by 7 patients.

The agmination of LyP-like papulonodules confined to a discrete circumscribed area is a distinct clinical subset within the spectrum of CD30⁺ lymphoproliferative disorders. The behavior of this entity is that of a progressive lymphoma that warrants therapy.