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 presents 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. Patients often report stress triggers 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 CD30-positive T-cells). The family of pcCD30+LPD includes diseases that are non-malignant and full-blown lymphomas. LyP is usually classified as non-malignant or as a cutaneous T-cell lymphoma (CTCL) precursor, though some experts consider it a very low-grade form of CTCL. 

The cause of LyP is not known. LyP is not contagious. There is no supportive research indicating that this is a genetic or hereditary disease. No single factor has been proven to cause this disease.

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)

Description
WHAT DOES LYMPHOMATOID PAPULOSIS LOOK LIKE?

LyP is characterized by pink or red-brown bumps, that may ulcerate, and can affect any area of the body. LyP lesions typically heal with scaling and crusting, and in some instances, scarring. Lesions usually heal over 2-3 weeks but may take as long as 8 weeks. The number of lesions can vary from each outbreak, and can vary in size and severity with each onset. Lesions may be asymptomatic or can be itchy or painful.

HOW COMMON IS LYMPHOMATOID PAPULOSIS?

LyP is a one-in-a-million disease, with only 1.2-1.9 cases per 1 million people. LyP onset can happen at any time in life – from early childhood to middle age, and affects all genders equally. Darker-skinned individuals seem less affected than other racial groups.

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)

Prognosis
WHAT IS THE PROGNOSIS OF LYMPHOMATOID PAPULOSIS?

The good news is that, in nearly all cases, LyP is a persistent but limited disease that does not affect a patient’s overall health. There is no known cure, but there are many treatments for living with the disease. Some cases resolve spontaneously.

Although LyP has an excellent prognosis, 10-25% of patients with LyP may be diagnosed with a second or associated T-cell lymphoma. Examples of this second lymphoma include mycosis fungoides (MF), cutaneous anaplastic large cell lymphoma (ALCL), or even systemic (internal) lymphomas.

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