 |
By Nikki with additions by Mike
Welcome to the weird world of lymphomatoid papulosis (“LyP” for short). My name
is Nikki. After two years of taking various antibiotics (which were not having any effect)
for a mysterious skin condition, I was finally diagnosed with LyP in March 1998. Since
you’ve found this page, chances are your dermatologist or general practitioner has
given you a diagnosis of LyP. You and your doctor(s) may have been trying to figure out
this strange skin condition for many months or many years (or in some cases, decades).
This rare condition has only been studied in depth since 1968 (Thanks to the work of now
retired dermatologist and clinician, Warren Macaulay, M.D. Lymphomatoid papulosis is often
referred to as Macaulay’s Disease), so don’t blame your doctors for their lack
of knowledge. Your doctor may have mentioned a link to lymphoma cancer, or he/she might
have already sent you to an oncologist for an examination. The good news is that most of
you will not have cancer. Unfortunately, some LyP patients have been misdiagnosed as
having a lymphoma cancer, and have been subjected to unnecessary surgery, and/or
chemotherapy and radiation treatments.
I will share with you information that I have gleamed from medical abstracts and medical
articles on LyP. Also available is a glossary of
medical word dictionary definitions you will run into, but might not understand.
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:
Click Here: Yahoo!
Groups : LyPSupport
LyP Resources
Books
Excellent but Technical: An
Illustrated Guide to Skin Lymphomas, Cerroni, Gatter, Kerl, 2nd. Ed,
2005
- Chapter 4: CD30+ Cutaneous Lymphoproliferative Disorders
- Lymphomatoid Papulosis, pp 45-51
Other Sites
Return to Other Lymphatic Cancers
and Conditions
Return to the Lymphoma Information Network
Text Copyright © 1998-2001 C.A. Thomason
Web Site Compilation and link organization Copyright © 1998-2005
Lymphoma Information Network - All Rights Reserved by both
http://www.LymphomaInfo.net/
Updated October 30, 2005 |
|
