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Lymphomatoid papulosis

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.

 

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