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Lymphomatoid Papulosis — SCE Dermatology MCQ

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HardSkin CancerLymphomatoid PapulosisSCE Dermatology

A 50-year-old man presents with lymphomatoid papulosis. He has recurrent crops of self-healing papulonodules on his trunk that resolve within weeks leaving varioliform scars. Biopsy shows large atypical CD30+ lymphoid cells with mixed inflammatory infiltrate. What is the prognosis?

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Correct answer: B — Excellent — benign clinical course despite atypical histology; 5-year survival >95%

Lymphomatoid papulosis (LyP) is a primary cutaneous CD30+ lymphoproliferative disorder characterised by recurrent crops of self-healing papulonodules. Despite alarming histology (large atypical CD30+ cells that can mimic anaplastic large cell lymphoma), LyP has an excellent prognosis with >95% disease-specific survival. However, approximately 10-20% of patients may develop a secondary lymphoma (MF, pcALCL, or Hodgkin lymphoma) over their lifetime, necessitating long-term clinical follow-up. Treatment is typically expectant management (no therapy) for mild disease, or low-dose Methotrexate for more symptomatic or extensive cases.

Reference: BAD 2019 CTCL Guidelines; EORTC-ISCL; WHO-EORTC