Lymphomatoid Papulosis

This piece was written by Guide Contributors
Stories of Help and Hope
My Personal Journey with Lymphomatoid Papulosis

NIKKI THOMASON
Santa Rosa, CA

My life has been transformed in many ways since I was diagnosed with Lymphomatoid Papulosis (LyP) over 19 years ago, and the changes have all been positive. These include increased self-confidence, new friendships, and fulfillment from helping others – all due to life’s unexpected twists and turns after acquiring a rare disease.

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Definition
What is Lymphomatoid Papulosis?

Lymphomatoid Papulosis (LyP) is a disease of the immune system that manifests with self-healing small bumps and spots on the skin that come and go. It can be persistent with frequent, recurring eruptions or it can disappear for an extended period of time before showing up again. Stress is often reported to trigger the breakouts.

LyP belongs to a family of conditions called primary cutaneous CD30-positive lymphoproliferative disorders (pcCD30+LPD). The name lymphoproliferative disorder is used to define a broad range of diseases of the immune system that share a common biology (in this case the presence of CD30-positive T-cells) and may span from non-malignant, inflammatory or infectious, all the way to full-blown lymphoma. In the family of pcCD30+LPD, LyP is usually classified as non-malignant or as a cutaneous T-cell lymphoma (CTCL) precursor, though some experts say it is a very low-grade form of CTCL. 

This is a one-in-a-million disease. LyP’s overall prevalence is only 1.2-1.9 cases per 1 million people. That said, onset can happen at any time in life – from early childhood to middle age, affecting both genders equally. Black-skinned individuals seem less affected than other racial groups.

How is LyP different from other cutaneous lymphomas?

EXPERT PRESENTER

Stuart Lessin, MD, Medical Director, KGL Skin Study Center (At time of publication: Dermatologist, Bryn Mawr Skin & Cancer Institute)

Diagnosis
How is LyP diagnosed?

LyP is diagnosed by a skin biopsy. The interpretation of the skin biopsy can be difficult and is best done by an experienced dermatopathologist. Patients who are diagnosed with LyP may expect additional studies such as skin biopsies, blood tests, X-rays and scans to detect any associated lymphomas.

Why are so many skin biopsies required?

EXPERT PRESENTERS:

Stuart Lessin, MD, Medical Director, KGL Skin Study Center, Broomall, PA

Pierluigi Porcu, MD, Director, Hematologic Malignancies and Hematopoietic Stem Cell Transplantation Division, Sidney Kimmel Cancer Center, Thomas Jefferson University Hospital (At time of publication: Associate Professor of Internal Medicine, Division of Hematology-Oncology, Ohio State University Comprehensive Cancer Center)  

Larisa Geskin, MD, Director, Cutaneous Oncology, Columbia University Medical Center (At time of publication: Associate Professor of Dermatology, University of Pittsburgh)

What does LYP look like?

LyP is characterized by red-brown bumps and spots, that may ulcerate and typically heal with scaling and crusting, and in some instances, scarring. Large plaques or nodules may occur but these are rare. Lesions usually heal over 2-3 weeks but may take as long as 8 weeks. The number of lesions can vary from each eruption and can vary in size and severity with each onset. Lesions may be asymptomatic or can be itchy or painful.

Is the cause of LyP known?

Is it contagious or inherited?

LyP is not contagious. There is no supportive research indicating that this is a genetic or hereditary disease.

What causes LyP?

Although there is continuing research, at this time, no single factor has been proven to cause this disease.

Treatment
What are the treatments for LyP?

Treatment depends upon the severity of the disease. In mild cases, with few lesions, a topical steroid cream may be used. Steroid creams do not prevent the development of new lesions but may be helpful in reducing the lesion symptoms. For widespread disease with many lesions, PUVA phototherapy, oral retinoids and low doses of metrotrexate (MTX) can be effective. In patients with LyP-associated lymphomas, treatment of the lymphoma will also clear the LyP in most cases.

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