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What is Lymphomatoid Papulosis?
Lymphomatoid Papulosis (LyP) is a disease of the immune system that manifests itself in self-healing papules and nodules (“bumps” and “spots”) that come and go spontaneously. LyP can be persistent with recurrent eruptions or it can disappear for weeks or months at a time before reactivating.
Who gets LyP and how common is it?
LyP is a one in a million disease ? the overall prevalence rate is estimated at only 1.2 to 1.9 cases per 1,000,000 population. The onset of LyP can happen anytime from early childhood to middle age, and it affects both sexes equally. Only black-skinned individuals seem to be less affected than any other racial group.
What does it 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. 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 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.
What is the progression of the disease?
In up to 90% of cases, LyP is a persistent or self-limited disease with no associations with other immune system diseases and does not affect the overall health of the patient. In up to 10% of LyP cases, there is an association with lymphomas, including progression to Hodgkin’s disease, cutaneous T-cell lymphoma (mycosis fungoides (MF)), or anaplastic large cell lymphoma. In patients with lymphomas, LyP may develop before, after or at the same time the lymphoma is diagnosed. Some experts consider LyP to be a low grade, self-limited form of skin lymphoma.
Is there a cure for LyP?
There is no known cure for LyP, however, there are therapies that are active in treating lesions.
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.
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 methotrexate (MTX) can be effective. In patients with LyP-associated lymphomas, treatment of the lymphoma will also clear the LyP in most cases.
What is CTCL?
CTCL is the acronym for cutaneous T-cell lymphoma. It is a general term for several lymphomas of the skin including mycosis fungoides, Sezary syndrome, cutaneous anaplastic large cell lymphoma, adult T-cell leukemia/lymphoma, peripheral T-cell lymphoma, and lymphomatoid granulomatosis. All cases of mycosis fungoides are CTCL, but not all CTCLs are mycosis fungoides.
What is mycosis fungoides?
Mycosis fungoides (MF) is an old term for the most common type of CTCL. It is a low-grade lymphoma that primarily affects the skin. Generally it has a slow course and often remains confined to the skin. Over time, in about 10% of the cases, it can progress to the lymph nodes and internal organs.
Is LyP a CTCL?
Most experts classify LyP as pre-CTCL and some classify it as a low-grade form of CTCL. The American Cancer Society does not classify LyP as a form of cancer, but it has been acknowledged as such in Europe. In 1997, the European Organization for Research and Treatment of Cancer (EORTC) formally classified LyP as a form of CTCL.
Where can I go for support?
Join the LyP Online Support Group by going to http://health.groups.yahoo.com/group/LyPSupport/. If you have any questions, contact the group moderator, Nikki Thomason at nikkithom@aol.com or contact the Cutaneous Lymphoma Foundation.
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