Hi porki-ga, and thanks for your question. As usual, this answer is
no substitute for medical evaluation or advice.
First, let me summarize your question... You describe four separate
disorders: Lymphedema (LE) of the face and neck, Lymphangioma (LA) of
the face and neck, Herpes Simplex II (HS2), and birthmark (Nevi
Flammei) on the leg. You are looking for possible connections between
these.
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Let me start where your symptoms started, your birthmark. There are
some congenital syndromes that can be associated with birthmarks, such
as Sturge-Weber Syndrome and Klippel-Trenaunay-Weber Syndrome, but
these typically have other features that you don't describe (such as
brain involvement, glaucoma, etc.). Birthmarks are very common, and
this is likely to be unrelated to the other problems you describe.
You can read more about these syndromes at the NIH Online Mendelian
Inheritance in Man database:
http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=185300
http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=149000
The American Academy of Dermatology has a basic discussion of the
various types of birthmarks here:
http://www.aad.org/public/Publications/pamphlets/VascularBirthmarks.htm
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Looking now at Lymphedema (LE) and Lymphangioma (LA), LE has many
causes and, as you state, is more typical in the extremities. There
have been cases of LE secondary to HS2 in the hand (known as Herpetic
Whitlow). In these patients, they usually had some type of open wound
and then came into contact with the HS2 virus and developed an
infection. The subsequent swelling blocks lymphatic drainage channels
resulting in LE. More on this later.
You may have come across some of the articles I will list, but since
I'm not sure what you've already read, I'll include them for
completeness since I've used them in my own research on the topics you
list.
Cervicofacial lymphangioma is congenital, but is only detected within
the first few months of life in about 50% of patients. The disorder
can go undetected in part due to its typical course:
"Slow progression followed by pseudoregression eventually gives rise
to reappearance. Rapid growth or engorgement with lymph or blood is
often associated with direct infection, trauma, or a secondary
respiratory or skin infection. Spontaneous regression has been
reported in as many as 15% of patients with cervicofacial
lymphangioma. Malignant change has not been reported."
This type of LA constitutes about 6% of benign tumors of childhood,
making it uncommon but not exceedingly rare.
You can read more on this topic at eMedicine.com. This page also
includes information on the current thought of how these malformations
form
http://www.emedicine.com/ent/byname/cervicofacial-lymphangiomas.htm
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LA is typically managed by an ENT surgeon (head and neck specialist).
eMedicine has a more detailed article on LA, which goes on to describe
the various subtypes of LA, where it tends to occur, and so on.
http://www.emedicine.com/ent/topic575.htm
In particular, this article states that:
"Lymphangioma in the head and neck generally involves a swelling or
mass that is soft to palpation and well circumscribed or diffuse. It
may have defined or ill-defined borders and is often associated with a
bluish discoloration."
"Regional venous congestion and the absence of pain are common."
In terms of diagnosis, the above article has a complete list of the
many means by which LA can be evaluated. As this site correctly
states, the ultimate diagnosis is made by tissue sampling. Fine
needle sampling generally doesn't yield the actual cells needed for
diagnosis, only the lymphatic fluid.
"Tissue diagnosis, as a direct and final correlate to imaging studies,
is the best and most consistent confirmation of lymphangioma."
These lesions typically present as neck masses, which must be
surgically removed. Unfortunately, they can have up to a 50%
recurrence rate. In any case, tissue from surgery can be sent to the
pathology lab for diagnosis if a sample cannot be obtain prior to
surgery (if the mass is too deep or too close to critical structures
for example).
There are some rare causes of LA, including Acquired Progressive LA
(also known as APL), with only 30 cases being reported worldwide
total.
http://www.emedicine.com/derm/topic848.htm
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There have been some cases of lymphangioma-like variants of Kaposi's
sarcoma (a previously rare type of cancer, prior to the HIV pandemic)
with associated LE. Here is a case report on this type of disorder:
"An HIV-positive black man developed subcutaneous noduli in the
framework of a lymphangioma-like variant of Kaposi's sarcoma.
Extensive lymphedema formation also occurred on the legs, face and
hands. Treatment with alpha-interferon led to partial regression of
Kaposi's sarcoma and the lymphedema."
Bossuyt L. Van den Oord JJ. Degreef H. Lymphangioma-like variant of
AIDS-associated Kaposi's sarcoma with pronounced edema formation.
Dermatology. 190(4):324-6, 1995.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=7655117&query_hl=18
There are other similar case reports in the literature. Only your
physician can determine if you may have this unusual type of disorder.
An HIV test would be an important first step if this is even a
possibility, since Kaposi's sarcoma is very rare in patients who don't
have HIV.
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In terms of relating LA to LE, one possibility is that LA, having a
mass effect, can obstruct lymphatic drainage resulting in secondary
LE. LE can also be present at birth. In this case, when it is a
congenital abnormality, one refers to it as Milroy Disease. In this
disorder, the lymphatic vessels fail to form correctly in utero. The
LE is present at birth and 70-80% of patients with Milroy Disease are
female. You can read more here:
http://www.emedicine.com/med/topic1482.htm
Another possibility is that LE can be associated with acquired LA.
Acquired LA is also known as Lymphangiectases. Here is an article
describing this phenomenon:
http://www.emedicine.com/derm/topic873.htm
Essentially, damage to the lymphatics from any of many causes (such as
radiation, scarring, etc.) causes dilitation of the superficial or
deep lymphatics.
"Lymphangiectases represent superficial lymphatic dilatation caused by
a wide range of scarring processes. Lymphangiectasia occurs as a
consequence of lymphatic damage by an external cause, leading to
obstruction of local lymphatic drainage. Lymphangiectases are also
termed acquired lymphangiomas. Acquired lymphangiomas most commonly
occur in adults as a late sequela of mastectomy and radiation therapy.
Patients usually present with numerous translucent vesicles in a
chronic lymphedematous area several years after surgery with or
without radiation therapy."
"Some authors apply the terms acquired lymphangioma and lymphangioma
circumscriptum interchangeably. In both conditions, the typical
cutaneous lesions are groups of small translucent vesicles, often
compared with frog spawn. Although both share similar clinical and
histologic features, the authors believe that they are 2 distinct
entities. The term acquired lymphangioma (lymphangiectasia) is used
when dilated lymphatic channels arise following damage to previously
normal deep lymphatics, whereas lymphangioma circumscriptum is used
when lymphatic channel dilation occurs because of congenital
malformations of the lymphatic system involving the skin and the
subcutaneous tissues."
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The mention of vesicles raises the question of possible connection
with HS2. HS2 typically presents with vesicles at the site of
involvement. If you are seeing vesicles in the head and neck area
involved with lymphedema, these are most likely to be the types of
dilated superficial lymphatics mentioned above. It's difficult to
rule out the possibility that if you had an active HS2 outbreak and a
skin wound (common in LE) in the head and neck area, then you may have
transmitted the HS2 virus to this area.
HS2 infects nerves and because of this follows dermatomal
distributions, for example in Herpes Zoster. If there is some
question as to whether or not the skin in the head and neck area is
involved with HS2, a scraping of a vesicle can be obtained and sent
for Tzanck staining, which detects the Herpes virus. Other detection
tests are also available (including PCR, which can distinguish between
Herpes Simplex I and II). You can read more, again at eMedicine, at
this page:
http://www.emedicine.com/med/topic1006.htm
You can see the dermatomal distributions mentioned above here:
http://courses.umassmed.edu/mbb1/2003/line_drawings/NE/NE-dermatome.gif
http://mywebpages.comcast.net/epollak/PSY255_pix/dermatomes-netter2.JPG
The only cases of Herpes causing secondary LE are in the arms or
hands, as I mentioned above. This is a well described potential
complication of Herpetic Whitlow, but I don't believe has been
described in the trunk or head and neck areas, possibly due to their
larger lymphatic drainage routes.
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So, to summarize...
A birthmark at birth isn't likely to predispose you to LA or LE unless
you have a rare syndrome, which it doesn't sound like you have.
Acquired LA (Lymphangiectases) can be associated with LE, depending on
the mechanism by which the LA was acquired and it's specific location,
etc. The best way to make the diagnosis is tissue sampling. A
distinction is made between Acquired Lymphangioma (Lymphangiectasia)
and Lymphangioma Circumscriptum, the former referring to (aptly) an
acquired condition sometimes due to radiation or other causes
typically involving the deep lymphatics, the latter being congenital
and more typically involves the skin and subcutaneous tissues.
HS2 is unlikely to be related to any of the above conditions, although
you may have a secondary infection, which you may be more prone to
with skin breakdown. Skin / blister scrapings could be tested to
determine if HS2 is present.
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I hope this information was helpful. Best of luck with the diagnosis
and treatment of your condition. Please feel free to request any
clarification.
-welte-ga |
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