What changed: Lymphomatoid papulosis

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The biopsy shows large atypical CD30-positive cells that on their own would suggest an aggressive lymphoma. What makes the diagnosis is the course: crops of papules that ulcerate, crust and heal on their own over three to twelve weeks, often leaving small scars, recurring over years. Photographs and a dated history are part of the diagnostic record, not an extra. Staging confirms there is no disease outside the skin.

No treatment has been shown to change the course or to reduce the risk of a second lymphoma, so treatment is for the lesions that bother the person. Observation with emollients and an explanation is a legitimate plan for somebody with a few lesions, and in the published series nobody has died of this condition. Where crops are frequent, numerous or scarring, low-dose weekly methotrexate, phototherapy or potent topical steroids suppress them, and the lesions return when treatment stops. Combination chemotherapy has no place.

The EORTC, International Society for Cutaneous Lymphomas and United States Cutaneous Lymphoma Consortium panel set out definitions, endpoints and treatment recommendations, and recorded that the level of evidence for most treatments is low.

In the Stanford series of CD30-positive skin lymphoproliferative disorders, no patient with lymphomatoid papulosis died of the disease, and overall survival was 92 per cent at five and ten years.

Macaulay described a self-healing eruption whose biopsy looked malignant and whose course did not, and called it lymphomatoid papulosis.