A category of cutaneous CD30 positive T cell lymphoproliferative disorder.
It is a benign, chronic, often relapse and condition belonging to the CD30 positive cutaneous lymphoproliferative disorders.
Chronic remitting skin disease with papules and nodules which typically regress in 3-6 weeks.
When large lesions are present complete regression may not occur leaving residual hypopigmented or hyperpigmented scars.
Biopsy reveals atypical T cell infiltrates.
Clinically benign cell killing skin papules and or nodules that have a histopathology of a high grade malignant lymphoma.
Diagnosis is often delayed, with persistent symptoms for one to 3 years before appropriate diagnosis.
Tumor cells derived from activated T cells that express CD30 antigen.
Disease specific survival at 5 years is 100% and overall survival at 5 years is 92% (Bekkemk MW).
Patients have an increased risk of developing a second neoplasm such as mycosis fungoides, primary cutaneous anaplastic large cell lymphoma and Hodgkin’s lymphoma.