SYNOPSIS
Primary cutaneous CD30+ lymphoproliferative disorders encompass lymphomatoid papulosis (LyP), primary cutaneous anaplastic large cell lymphoma (pcALCL) and indeterminate cases. LyP is a benign disorder characterized by recurrent crops of several to hundreds of papulonodules, red or violaceous in color and measuring up to 20mm, usually on the trunk and extremities. Patients with LyP are at increased risk of a secondary malignancy that may be diagnosed before, during or after the diagnosis of LyP and thus should receive ongoing surveillance. pcALCL is characterized by a solitary red to violaceous nodule or tumor greater than 20mm and may occur anywhere on the body. Secondary cutaneous ALCL must be excluded for any patient initially presenting with a cutaneous lesion of ALCL. LyP is benign, limited to the skin and self-resolves with a 5-year survival rate of 100%; pcALCL is usually limited to the skin and responsive to directed therapies, with a 5-year survival of over 95%. Aggressive systemic or multi-agent chemotherapeutic regimens should be avoided.