Lymphomatoid Papulosis

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Lymphomatoid Papulosis

Postby patoco » Sat Jun 17, 2006 4:59 pm

Lymphomatoid Papulosis

Lymphedema People

http://www.lymphedemapeople.com

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Lymphomatoid Papulosis

Here is one medical definition of lymphomatoid papulosis:

Clinically benign, histologically malignant, recurrent cutaneous eruption characterized by an infiltration of large atypical cells surrounded by inflammatory cells. The atypical cells resemble Reed-Sternberg cells of Hodgkin’s disease or the the malignant cells of cutaneous T-cell lymphoma. In some cases, lymphomatoid papulosis progresses to lymphomatous conditions including mycosis fungoides, Hodgkin’s disease, cutaneous T-cell lymphoma, or anaplastic large cell lymphoma.

Whereas the American Cancer Society did not classify LyP as a cancer, in 1997 the EORTC (European Organization for Research and Treatment of Cancer) published a classification of cutaneous T cell lymphomas (CTCL) which included lymphomatoid papulosis as a low grade CTCL.

At the 88th Annual USCAP Conference in San Francisco, CA (3/21/99) Dr. Marshall Kadin stated there are 3 types of LyP:

Type A contains CD30+ large cells (immunoblasts) and numerous inflammatory cells - small lymphocytes, neutrophils and eosinophils which impart an appearance resembling that of Hodgkin's disease in lymph nodes.
Type B comprises mostly smaller cells with irregular or convoluted nuclei resembling tumor cells of mycosis fungoides; there is often epidermotropism and the distinction from MF [Mycosis Fungoides] is clinical.
Type C contains large numbers or sheets of immunoblasts but is largely confined to the dermis which distinguishes it from anaplastic large cell [Ki-1] lymphoma.
In layman’s terms, LyP is a rare (from a 1992 article: the overall prevalence rate of lymphomatoid papulosis is estimated at 1.2 to 1.9 cases per 1,000,000 population and may occur less frequently in black patients) lymphatic system disorder consisting of self healing nodules and papules (you or I would call them "bumps" or "spots") that “wax and wane”. In other words, if you leave them alone, they eventually go away by themselves. Some lesions are small, round, shiny and smooth. Other are large, open to the air, gooey and have a crust in the middle. Some leave scars and some do not. Some leave flat scars and some leave keloid scars. The colors range from pinkish red to pinkish brown and purple. If you would like to see photos of LyP skin lesions please check out DermIS.

The number of lesions present can vary from one to hundreds. Crops of lesions may come and go for decades. At this time there is no cure for LyP.

Most doctors treat their patients with a topical steroid cream (Diprolene comes to mind) and/or PUVA (psoralen - an orally ingested photosensitizing drug combined with ultraviolet A light exposure). Some doctors prescribe very low oral doses of a chemotherapy drug called Methotrexate.

No familial cases have been reported (in other words, you don’t get it from your parents or your grandparents, and you can’t pass it onto your children).

If you are interested in reading medical abstracts on LyP online please check out PubMed.

The following article is the most complete I've read on LyP so far. My dermatologist got a copy for me (Thank you, Dr. Michael Haiman of Kaiser Permanente!). Ask your doctor if he will do the same for you.

Journal of the American Academy of Dermatology
Volume 30 Number 3 March 1994

Continuing medical education
Lymphomatoid papulosis

Debra Lynn Karp, MD, and Thomas D. Horn, Baltimore Maryland

Update: There is a good book that has come out in October, 2005 An Illustrated Guide to Skin Lymphomas, Cerroni, Gatter, Kerl, 2nd. Ed, that devoted most of chapter 4 to LyP.

Okay, I know you are wondering about the lymphoma cancer link. Most papers on LyP suggest that 10-20% of LyP patients go on to develop an associated CTCL (cutaneous t cell lymphoma). My advice to you is not to dwell on what might or might not happen in the future. Try to live a healthy lifestyle and think positive (some doctors and patients believe that stress increases the amount of lesions). If you do develop symptoms of lymphoma (swollen lymph nodes, fever, unexplained weight loss, night sweats) contact your doctor.

If you have LyP (as verified by your doctor’s diagnosis and a biopsy slide and report from pathology), I would strongly recommend that you join the Lymphomatoid Papulosis Central Registry at Beth Israel Hospital in Boston, MA.

In my biased opinion, if anyone is going to find a cure for this condition, it will be hematopathologist Marshall E. Kadin and his colleagues. (On March 1, 1999, Dr. Kadin wrote in an e-mail: There are two LyP funds. One is at my hospital and the other is in Fargo, North Dakota, organized by Warren Macaulay, the man who first described LyP. His fund is supporting a research project in my lab to look for a possible virus in LyP. We are making the first rigorous investigation of this possibility and should know the answer within a year). You can e-mail Dr. Kadin at mkadin@caregroup.harvard.edu to get more information about joining the registry or to learn about his LyP clinical trials.

I was unable to locate an online support group for folks with LyP, so I started an electronic mailing loop in September 1998. This allowed folks to submit group posts to "discuss" what's happening in our lives and the medical research community in regard to this disorder. We have now switched over to a mailing list, which makes it easier for folks to post to our discussion group. If you (or a family member) has LyP and would like to join a wonderful group of folks from around the world, please go to the Yahoo Groups site below. The name of the list is LyP Support; you can learn more about the list by clicking on the button below:

Lymphoma Information Network

http://www.lymphomainfo.net/nhl/types/lyp.html

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Lymphomatoid Papulosis

I. Definition:

Lymphomatoid papulosis is a stubborn persistent rash that usually occurs on the chest, stomach, back, arms and legs. The rash often consists of red or brown bumps that have a crusty surface. The bumps heal in two to three weeks but new ones continue to form. This condition is slightly more common in women.

II. Causes:

The cause of lymphomatoid papulosis is not known.
In some people lymphomatoid papulosis turns into lymphoma which is a form of cancer. This happens in about one out of ten people.
Lymphomatoid papulosis often lasts for years. Patients need to see their doctor regularly to make sure their condition does not turn into cancer.

III. Treatment:

Lymphomatoid papulosis is difficult to treat and there is no cure for this condition.

Cortisone ointments will sometimes help.
Ultraviolet light treatment will help control lymphomatoid papulosis.
Methotrexate is sometimes used to treat lymphomatoid papulosis.

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Support

LyPSupport · Lymphomatoid Papulosis (LyP)

Yahoo Groups

http://health.groups.yahoo.com/group/LyPSupport/

LyP Registry

http://www.infoderm.com/support/lymphoma.html
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