With anyone male or female that has lymphedema of the lower limbs, genital lymphedema is a very real possibility. This is especially true of those whose lymphedema expresses itself early in childhood.
While this subject may embarrass some, there needs to be discussion and enlightenment on it.
Related terms genital lymphedema, lymphedema of the scrotum, lymphedema of the penis, scrotal edema, scrotal lymphedema, labia swelling, vulvar lymphangioma, genital oedema, lymph scrotum, male genital lymphedema, vulvar lymphedema, Crohn's Disease, Buck's Fascia, lymphedema of the externa genitalia, genital lymphedema in children
Like lymphedema that affects any body part, genital lymphedema is either primary or secondary.
Causes include infections (cellulitis, lymphangitis; lymph node removal for cancer biopsy; trauma or injury; lymphoceles; parasystic infection; cancer treatment; sexually transmitted disease ( lymphogranuloma_venereum)
The major complication from genital lymphedema is cellulitis. Other complications include loss of sexual function, infertility (depending upon the severity) difficulties in urination. Other complications include sever social stigma, skin rashes, fungal infections.
For female genital lymphedema patients have almost no options available. Unlike male genital lymphedema there are no surgical options that I am aware of. This leaves only manual decongestive therapy and the wearing of compression Garments.
Management also includes diagphramtic breathing, exercise and weight control.
(TMER) for patients with Genital Lymphedema. Our Deepest appreciation to Denise from
St. Ann's Hospice Lymphoedema Clinic
and to Silkie for obtaining this for us!
Choose a time each day to carry out this massage and exercise programme when you can lie on the bed and relax while you are doing it. You will also need to remove and or loosen any clothing which would get in the way of the massage.
Your skin should not look red or pink at the end of the massage- if it is you must be pressing too hard- go lighter.
1. Lie with your knees bent up, feet on the bed. Place both hands on your stomach just below your ribcage. Breathe in as deeply as you can through your nose so that the air pushes your stomach up under your hands. Then breathe out through your mouth, pulling your stomach muscles in at the same time to squeeze all the air out. Repeat 5 times
2. Place one arm above your head, place your other hand just below your arm pit and gently and slowly move the skin round in as big a circle as possible with your hand. After approximately one minute change and repeat the same routine under the other arm. For approx. 1 minute on each side.
3. Using both hands stroke gently and very slowly from your groins on both sides up towards your armpits. Then stroke from the centre- just above your genital area, up and out towards your arm pits You can do both sides at the same time, or just one side at a time which ever is easier. When massaging try to make sure that your hands are relaxed and the whole hand is in contact with the skin.
Try also to massage your back from the central crease between your buttocks up over your waist-line or ideally get somebody else to help you with this. Massage for at least 2-3 minutes on each side.
4. Place your hands in your groins and as you did in ‘2’, slowly move the skin round in as big a circle as you can. For approx. 1 minute
5. With your knees bent up, squeeze your buttocks together as firmly as possible hold this while you tighten the muscles of your pelvic floor between your legs and then pull in your stomach muscles as hard as you can- hold them tight all together- and then relax. Imagine you are trying to zip up a really tight pair of jeans and having to pull everything in to get the zip to close. As you tighten everything up, breathe out- as you relax, breathe in. Repeat 5 times.
6. Hip and knee exercise. Bend your knee up towards you. Clasp your hands round your thigh and gently pull your knee towards your chest hold it there for a count of 2 then release the pressure by straightening your elbows repeat this slowly a further 4 times. Change legs and go through the same routine on the other side.
7. Finish off with an ankle exercise. Pump each foot up and down at the ankle, slowly and deliberately, 20 times.
This combination of exercise and massage will generally improve the lymphatic drainage from your lower body. Movement and exercise always helps to stimulate lymph drainage. Try not to sit for long periods without movement, keep exercising the muscles of your pelvic floor it will help. You may need advice from a physiotherapist about this.
Care of the skin of your lower body and genital area is just as important as of the legs.
Moisturise with a very bland cream such as Aqueous Cream, gently massaging any very firm areas of swelling to soften them. This is best done after the massage and exercise routine above, so that you have cleared the way ahead for lymph to drain. .
Use Aqueous Cream to wash the genital area instead of soap, it is less drying and will reduce irritation.
Always dry very carefully in skin creases and folds and don’t let cream accumulate in them. Too much moisture in the creases encourages fungal infections. If the skin in the creases looks red and irritated, consult your doctor, you might need an anti-fungal cream.
Any infection can make the lymphoedema worse and needs prompt attention- particularly cellulitis.
SUPPORT for the genital area to reduce swelling can be helpful. Some of the hosiery companies do make garments rather like cycling shorts to provide compression in this area. But sometimes buying lycra firm support panties with legs in and placing a pad inside to put additional pressure on the genital area gives further support. Obviously it is important that they don’t constrict the lymph drainage from the legs.
Manual Lymphatic Drainage Massage (MLD) can be helpful with genital oedema. Find out if this is available from your nearest Lymphoedema Clinic. It is available privately in some areas- lists of practitioners are available from MLD UK © Copyright 2005 by Pat O'Connor and Lymphedema People. Use of this information for educational purpose is encouraged and permitted. It must be available free and without charge and not used for financial renumeration or gain. Please include an acknowledgement to the author and a link to Lymphedema People. All links associated with this article must be listed as well.
Very Special Thanks to Cheri Hoskins (Healthronix)and Cyndi Ortiz for their response to questions posted on our discussion boards
Cheri L. Hoskins, CCT President Healthtronix Lymphedema Management, Inc.
It is very difficult to answer questions of this nature without seeing someone or having a history (iteology, etc). However, there are a few tricks that you can try biker shorts worn daily with compression in the crotch, Elvarex (a Jobst product) makes a wonderful product, just make sure when you are measured the therapist knows that you need compression in the crotch.
When sitting, sit in a reclined position so as to relax the inguinal nodes and prevent a crimp in that very important drainage area.
In addition, do deep breathing exercises several times a day to create a vacuum in your abdomen.
© Copyright 2005 by Pat O'Connor and Lymphedema People. Use of this information for educational purpose is encouraged and permitted. It must be available free and without charge and not used for financial renumeration or gain. Please include an acknowledgement to the author and a link to Lymphedema People. All links associated with this article must be listed as well.
By Melanie Lewis MCSP SRP, Macmillan Lymphoedema Clinical Specialist Service Co-ordinator
Lymphoedema of the genital region is relatively uncommon, but is extremely uncomfortable and distressing for the patients who suffer with this condition. It can affect both men and women alike, but is seen more frequently in males due to the anatomical differences between the genders and effects of gravity. Around ten percent of people who develop leg oedema will have associated genital swelling, but some patients can have genital oedema alone.
In some circumstances, genital oedema can occur acutely due to trauma or cellulitis and may be able to resolve completely by itself. Far more usual however, is the chronic genital oedema, which is unfortunately irreversible, but can be controlled and reduced through appropriate lymphoedema management. The main cause of genital oedema is either due to primary or secondary lymphoedema.
Primary lymphoedema affecting only the genitals is rare. It can be noticed from birth or during the teens, and as the affected individual grows, the involved lymphatic system becomes ever more under pressure to drain the tissue fluid and the swelling becomes far more obvious. The main reasons for primary genital lymphoedema are that the lymph vessels are absent or reduced in number or simply don't work as well as they should i.e. functional failure. It has also been thought that primary lymphoedema patients who are obese, have an increased risk of genital swelling due to greater pressure on the groin from the enlarged abdomen.
Secondary lymphoedema more commonly affects the genital region than primary lymphoedema. In Africa, India and other tropical countries, genital swelling is frequently seen due to infectious diseases like filariasis. This can lead to gross elephantiasis of the penis and scrotum. In the Western world, the majority of genital oedemas are from trauma or surgery to remove gynaecological, urological, abdominal or prostatic cancers. It has been reported that up to 70% of patients treated for carcinoma to the vulva will have lower body swelling. Radiotherapy to the lymph nodes in the groin or abdominal region can also cause genital lymphoedema. The incidence also increases if there has been surgery and radiotherapy plus episodes of cellulitis.
Various parts of the genital anatomy can become swollen. In males, both the penis and scrotum, or each, can swell independently. Very few patients just have penile oedema, but it does happen, as can be seen from the case study. Sometimes, the scrotum becomes so swollen, that the patient has difficulty in walking. As the swelling increases, it can involve the area above the base of the penis (called the pubic area), thus causing the penis to retract into the scrotum. This clearly causes problems for micturition (urination)and sexual activity.
In females, the inner and outer lips of the vagina (labia) can become so swollen that they extend out of the vagina by up to 6 inches; again this creates problems for sexual activity and urination. In both genders, the pubic area on the lower abdomen alone can become oedematous, with associated skin changes and fibrosis.
Genital swelling can occur due to other causes. Palliative patients who have renal, cardiac or hypoproteinaemia (high output failure due to low protein) and patients who have had venous problems, could develop genital oedema. A clear diagnosis and medical investigations are needed, prior to lymphoedema management.
Pain is a problem for some patients, who describe a dragging, heavy, bursting sensation or an ache around the genital region. This is usually eased when the area is decongested or lifted by a jock straplike support or cycling shorts.
Skin changes are readily seen in genital oedema. Thickening and dry, flaking skin (hyperkeratosis) or warty blisters (papillamatosis) do occur as the swelling progresses.
Infections are commonly seen in oedematous skin, which is the ideal medium for bacteria as it is generally warm, moist and has numerous crevices. The bacteria multiply in the protein rich oedema fluid, and infections can spread throughout the genital region, causing it to be red, hot, tender and swell even further. More often than not, an infection is seen as the precipitating factor in causing the swelling.
Fungal Infections do occur, due to the area being moist, warm and having so many crevices. Sweating also can trigger fungal infections.
Lymphorrhea occurs when the tissue pressure increases and causes leakage of fluid from the thin layer of skin. Lymphorrhoea can continue for a few days or weeks and carries a high risk of developing infections. It can be very distressing for patients, as some have to wear incontinence/sanitary pads to absorb the copious fluid. Lymphoedema treatment is necessary to stop this leakage.
Sexual Dysfunction happens as the oedema increases. In males, impotence or painful erections impede sexual intercourse. Females find that the presence of oedema dampens sexual activity, due to decreased libido and pain.
Sexual Dysfunction happens as the oedema increases. In males, impotence or painful erections impede sexual intercourse. Females find that the presence of oedema dampens sexual activity, due to decreased libido and pain.
The four cornerstones of lymphoedema care can be modified to treat genital oedema. Skin Care and meticulous hygiene of the genitals is imperative. Daily bathing with an antibacterial soap and drying the area afterwards is very important to reduce the likelihood of infections. Regular moisturising with an aqueous cream will deter any areas of dry, flaky skin and keep the area soft. As this area is prone to fungal infections and cellulitis, regular inspection will enable the patient to detect any early signs of inflammation. If an infection occurs, prompt anti- fungal or antibiotic treatment is required. If a patient suffers from recurring cellulitis episodes, then longterm prophylactic antibiotics may be required.
Compression Garments or Multi- Layered Bandaging techniques are needed to give the genital area support and compression. The penis, scrotum and labia areas will continue to swell until a firm outer casing prevents them from doing so. This outer casing works by providing the muscles with a base to press against, thereby, reducing the swelling.
The best form of compression garment comes in the form of custom-made tights or shorts. Spandex or padded cycling shorts and sports jock straps are also very useful to provide more comfort to the oedematous areas. Under garments must be firm and supportive, not loose. In some instances, two pairs, or an under garment plus swimming trunks, have been found to be effective.
Foam inserts also can increase the amount of compression to the penis, scrotum or female genital area. Ladies may find that the addition of a sanitary towel underneath garments is also helpful. For male patients with significant penile and scrotum swelling, a regime of multi-layered bandaging may be appropriate. This will consist of washable or disposable bandages and padding/foam being applied to the scrotum and penis separately. Your lymphoedema specialist will need to have had additional training in managing lymphoedema of the genitals, as bandaging the genital area can be very awkward, particularly in getting the bandages to stay in place once the oedema has reduced.
Occasionally, bandaging can cause an irritation at the base of the penis and the edge of the scrotal bandaging, thus care must be taken to ensure adequate padding is in place.
Simple solutions that have helped, include creating a harness for the swollen scrotum, using a soft pliable material like splint foam or 'Velfoam' prior to padding and bandaging. The harness creates more uplift for the scrotum and patients find it more comfortable as the bandages don't tend to slip. The harness and the penile bandaging can be kept in place using Velcro strips, as it is much easier to apply and reapply and does, therefore, tend to stay in place better. The use of compression shorts, post bandaging, also draws the genitals close to the body and also keeps the bandages in place. All bandages can be easily removed for micturition or if soiled, and the patient taught how to apply/reapply them. The use of bandages can significantly reduce the oedema, which would be maintained by compression garments such as shorts or tights.
Exercise in any form is important, as it keeps all the joints and muscles working adequately. If there are no areas of broken skin, then an excellent form of exercise is swimming or walking in the water. The genital area will have some support from the swimming attire and the pressure from the water assists too. Other forms of aerobic exercise that are also useful are cycling and walking, but it is important that compression garments and padding are worn when cycling.
A specific form of exercise for female genital oedema is the pelvic floor exercise. Together with abdominal exercises and diaphragmatic breathing, pelvic floor exercises can help in reducing the oedema. Ask your lymphoedema specialist or physiotherapist for further advice.
Lymph Drainage is an important part of lymphoedema management. Manual Lymphatic Drainage (MLD) and Simple Lymphatic Drainage (SLD) are massage techniques designed to move fluid away from the swollen genital region, to parts that are not affected, to drain freely. The massage itself is very light and is not painful. It is also very useful in softening hard, fibrosed tissue. MLD is a technique that is carried out by trained therapists. SLD is a simplified form of MLD and can be taught to the patient or carer to do themselves.
In some cases where conservative treatment does not control the swelling, surgical intervention may be required. Surgery could involve reducing the scrotum, penis or labia with the aid of plastic surgery skin grafting.
Mr A is a 68-year-old gentleman who has suffered with genital oedema since November 2001.Whilst on holiday in 2001, Mr A developed a painful spot on the right buttock possibly from an insect bite. Unfortunately, this blemish continued to increase in size and eventually became an abscess. He was operated on 3 times in a generalist hospital and due to infections and gangrenous tissue, some of his inguinal lymph nodes were removed. Mr A's genital swelling started soon after the surgery and was sited in the penis area alone. He unluckily had numerous cellulitis episodes, which in turn increased the penile swelling. The scrotum area was severely distorted due to the previous operations and in December 2002, Mr A underwent plastic surgery to graft and lower the testicle area, which although improved the cosmetic appearance of the testicles increased the penile swelling.
Mr A was referred to the lymphoedema service and assessed in June 2003. On examination, the genital area was red, inflamed and had a discharge from the shaft of the penis, which was grossly oedematous. The lymphorrhoea had been present for the last 6 months and Mr A had to pad the area to stop it staining his under garments. Severe skin changes were apparent with brown discolouration patches, hyperkeratosis and fibrosis all over the penis. The pubic area was also swollen and fibrosed.
Functionally, Mr A felt all forms of activity were limited, as well as travelling and socialising. He suffered an extreme amount of discomfort and pain, which hindered his mobility, and psychologically he felt that the oedema had greatly affected his quality of life and the way in which he viewed himself as a man.
Treatment commenced immediately, with Mr A starting a 2-week course of antibiotics to manage the infection. Information regarding hygiene and daily moisturising with an aqueous cream was initiated to help the skin changes, and antibacterial talc was recommended to reduce friction in the groin region. A simple technique of bandaging was also taught to the patient to reduce penis size and stop the leaking fluid. MLD was started and SLD was taught, to improve the fibrosis and create collateral drainage.
Mr A was reassessed four weeks later and was delighted with the results. His penile swelling had reduced significantly, making it look far more normal. The skin condition was greatly improved with all areas of hyperkeratosis and leaking diminished. His mobility was normal due to the pain being relieved and he informed me that he had booked a holiday. He is continuing with his lymphoedema regime, consisting of SLD, multi-layered bandaging and daily use of his compression padded cycling shorts, which will keep him in control of his genital oedema.
OB/GYN News, August 1, 2003, by Kate Johnson
MONTREAL – Unusual gynecologic complaints of labial swelling or clear labial/vaginal discharge could be symptoms of genital lymphedema, especially if the patient has had previous pelvic or gynecologic surgery or radiation affecting lymph nodes or vessels.
Awareness of this phenomenon is slowly growing among gynecologic oncologists, but until recently “there was [little recognition] that gynecologic lymphedema could complicate their treatments,” Dr. Andrea Cheville, director of the University of Pennsylvania Cancer Center's Lymphedema Program, said at a meeting sponsored by the World Federation for Ultrasound in Medicine and Biology.
“I have found limited receptivity regarding lymphedema on the part of gynecologic oncologists. This reflects the general emphasis in cancer care on disease and worrying about recurrence. Historically, there hasn't been emphasis on addressing the non-life-threatening sequelae,” she said in an interview.
Lymphedema can occur after treatment of gynecologic malignancies such as ovarian, endometrial, or cervical cancer, because of the extensive use of pelvic lymph node dissection and radiation therapies. In addition, treatments for bladder, colon, and renal cancer also have potential to compromise the deep lymphatic structures, increasing the risk of gynecologic lymphedema, she said.
The incidence of genital lymphedema is not known, largely because it often goes undiagnosed, but it has been estimated to occur following 10%-20% of all gynecologic oncology surgery and radiation therapy. Like other forms of lymphedema, it most commonly occurs in the first 3-4 years after cancer treatment, but can occur up to 30 years later.
“For patients with this history, if they have any genital swelling; changes in the skin texture; changes in hair growth; thickening of the labia; the presence of papillomas or discreet warty growths; or lymphorrhea, which is leakage of serous fluid through compromised or intact skin, think lymphedema,” she said.
Lymphorrhea may be difficult to recognize, especially if it is occurring intravaginally, but physicians can distinguish it from normal vaginal discharge or vaginal infections in a number of ways. “Many times vaginal discharge is whitish or curdish, thick, and opaque, but this is not. Lymphorrhea tends to be clear or a little bit yellow colored. If you culture it, it will be negative. But patients may sometimes complain that it is malodorous. Lymph has no odor, but it is very proteinaceous, which makes it a good culture medium for bacteria,” she said.
Genital lymphedema is a devastating condition, but unlike breast cancer, it is not a topic of polite conversation, Dr. Cheville said. She urged physicians to ask patients about these symptoms.
Treatment for the condition, as with general lymphedema, involves combined decongestive therapy consisting of compressive bandaging and manual lymph drainage, but this treatment approach can prove very problematic in gynecologic lymphedema.
“Bandaging is very difficult, because it's tricky to adequately compress the vulvar region,” she noted, adding that she uses a specially designed bandage with Velcro straps and odor control pads.
She recommended that unless physicians have training in lymphedema management, they should refer the patient, but she acknowledged the difficulty in finding well-trained therapists.
“There are very few therapists who have comfort and experience treating genital lymphedema. Predominantly these would be physical therapists, but some nurses and some occupational therapists do it as well.”
Fadare O, Brannan SM, Arin-Silasi D, Parkash V.
Department of Pathology, Vanderbilt University School of Medicine, Nashville, TN 37232, USA. email@example.com
This report describes 2 cases of localized vulvar lymphedema that were diagnosed in 2 morbidly obese women, including a unique case of a massive localized vulvar lymphedema associated with lymphangioma circumscriptum of the vulva. Herein, we also review previously reported cases of vulvar lymphedema and discuss differential diagnostic considerations. Our review of the recent literature showed 17 cases that we considered approximately similar, reported under the appellations “vulvar lymphoedematous pseudotumor,” “massive vulval edema,” “vulvar hypertrophy with lymphedema,” and “localized lymphedema (elephantiasis).” The patients ranged in age from 19 to 59 years (average 38.5 yrs), and typically presented with papillomatous plaques, skin polyps, generalized vulvar enlargement, or massive pedunculated masses that had been present for durations that ranged from 3 months to 36 years. The average lesional size was 6.1 cm (range: 0.6 - 45 cm), and 10 (59%) of the 17 cases were 3 cm or less. Excisions were generally curative, although persistent or recurrent disease was reported in 3 cases. The patients were overweight in 9 (75%) of the 12 cases in which the patient weight was noted, and 2 others had chronic immobilization. Stromal edema was the only morphologic finding that was uniformly seen in all 17 cases. However, the following morphologic features were identified in significant subsets: multinucleated giant cells, dermal fibrosis, dermal lymphangiectasia, dermal chronic inflammation, perivascular chronic inflammation (superficial and/or deep), hyperkeratosis, acanthosis, and blood vessels of varying calibers. Several attributes of localized vulvar lymphedema may cause them to closely mimic aggressive angiomyxoma, a differential diagnosis that is herein discussed in detail. Localized vulvar lymphedema may also be a small lesion, and can potentially mimic other myxedematous tumors of the vulvovaginal region. The strongest clinical association is with obesity. The term “localized vulvar lymphedema” is an appropriate generic descriptor for the spectrum of lesions whose fundamental and underlying etiology is thought to be chronic lymphedema.
Servicio de Dermatologia. Hospital Universitari Vall dHebron. Barcelona.
Cutaneous-mucosal lesions constitute one of the most frequent extraintestinal manifestations of Crohn's disease and in some cases may be the first symptom of intestinal disease. We describe the case of a 45-year-old female patient who sought medical help for genital tumefaction of 20 years' evolution. For the previous 15 years, she had been experiencing digestive symptomatology attributed to irritable bowel syndrome. Two months before the consultation, and coinciding with aggravation of the condition, the patient had been diagnosed with colonic Crohn's disease. Skin biopsy of the labia minora revealed sarcoid granulomas. The results of microbiological studies (staining for microorganisms and cultures) were negative. A diagnosis of metastatic vulvar Crohn's disease was made and, treatment with metronidazole was started, which improved the genital edema after 2 months. Genital lymphedema is an exceptionally rare manifestation of metastatic Crohn's disease that may appear several years before intestinal symptomatology develops. Treatment with metronidazole seems to be a good therapeutic option. Pub Med
Department of Urology, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA.
PURPOSE: This article presents a simple classification of lymphedema of the external genitalia, which is useful for selecting the appropriate therapy, and evaluates our experience with the various therapeutic options used to treat this disorder.
MATERIALS AND METHODS: The literature was reviewed and the records of patients treated for the disorder were analyzed. RESULTS: A convenient classification of the disorder divides cases into congenital and acquired. Therapy is primarily dependent on whether the disease is self-limited and whether there has been any pathological change in the skin, lymphatics and subcutaneous tissue. For self-limited diseases in which no permanent pathological sequelae occur conservative therapy is appropriate. For most chronic conditions a surgical procedure is required. Excisional techniques are most effective for severe forms of the disease. In select cases subcutaneous tissue excision with preservation of the overlying skin is appropriate. However, for most patients excision of the skin and subcutaneous tissue with split-thickness grafting is most effective.
CONCLUSIONS: When patients with lymphedema of the external genitalia require surgery and are properly selected for the appropriate procedure, the functional and cosmetic results are excellent and patient rehabilitation is likely. Pub Med
Vang R, Connelly JH, Hammill HA, Shannon RL. Department of Pathology and Laboratory Medicine, University of Texas Medical School, Houston, TX, USA.
We report the case of a 43-year-old quadriplegic woman with bilateral vulvar enlargement. The clinical impression was labial hypertrophy, but the microscopic features mimicked aggressive angiomyxoma because of the location, hypocellular proliferation of fibroblastic cells in an edematous-myxoid stroma, and vessels with perivascular collagen deposition, which simulated the thick-walled vessels of aggressive angiomyxoma. Since the lesion lacked true thick-walled vessels and contained ectatic tortuous lymphatics, the pathologic interpretation was lymphedema. This vulvar lesion should be recognized to prevent the misdiagnosis of aggressive angiomyxoma.
Lymphedema has not received much attention in the literature in the form of pathologic descriptions. It may rarely cause clinical problems as an enlargement or mass. Farshid and Weiss1 recently reported a series of cases in which lymphedema may cause a localized extremity mass and create microscopic confusion with well-differentiated liposarcoma or fibromatosis. Clinical hypertrophy of the labia (vulvar), in which the histologic consideration is aggressive angiomyxoma (AA), has not been documented. Lymphedema involving the vulva has the potential to result in a worrisome microscopic diagnosis if the proper clinical information is not given or if the pathologic spectrum of lymphedema is not understood.
REPORT OF A CASE The patient is a 43-year-old quadriplegic woman with a history of urinary bladder calculi, managed with an indwelling Foley catheter. Her quadriplegia, which she has had for 17 years, was the result of a motor vehicle accident. She was admitted for reconstruction of bilateral labial hypertrophy. The only other significant medical history was of repeated repositioning of the urinary catheter. The labial hypertrophy was thought to be related to her cord injury, with deformation of the labia by stretching and trauma over the years; the clinical impression was not that of a neoplasm. A labial reconstruction with resection of the bilateral hypertrophied areas was performed, and this vulvar specimen, designated as “hypertrophy-masses,” was submitted for pathologic examination. The right labial tissue measured 3.4 × 3.2 × 1.5 cm, and the left, 3.7 × 3.5 × 2.1 cm. The external surfaces were covered by gray-tan wrinkled skin. The cut surfaces were light tan, almost white, and soft.
PATHOLOGIC FINDINGS All sections from both sides of the vulva were uniform throughout. The overlying squamous epithelium was intact and showed no pathologic alterations. The dermis was expanded by a diffuse, uniform, and hypocellular proliferation of spindled and stellate cells. There was a moderate amount of lightly eosinophilic cytoplasm, and cytoplasmic borders were ill-defined. The nuclei were oval to elongated and had bland chromatin. No nucleoli, mitoses, or significant atypia were seen. The stroma was edematous to myxoid, with the more superficial aspects predominantly myxoid. The more edematous regions of stroma contained thin and wavy collagen fibrils. There were numerous small round blood vessels with perivascular collagen deposition, but no thick-walled vessels or red blood cell extravasation was evident. Occasional variably sized, ectatic, and tortuous lymphatic spaces were present There were occasional small clusters of capillaries with mild perivascular lymphocytic inflammation. Nerve fibers showed mild perineural edema.
COMMENT Clinical hypertrophy of the labia (vulvar) has limited histologic depictions and has not included the microscopic changes seen in this patient.2 The case presented here, which simulates AA, is designated “vulvar hypertrophy with lymphedema.” The term incorporates the clinical entity, while also retaining the pathologic basis for the histologic presentation.
The clinical impression in our case, bilateral labial hypertrophy, was inconsistent with AA. Aggressive angiomyxoma is a deep lesion, and the abnormalities should not be as superficial as those in our case. Taken out of context, the diffuse proliferation of low-grade fibroblastic cells in an edematous-myxoid stroma in the vulva mimics AA. The clue to the correct diagnosis requires attention to the vasculature. Aggressive angiomyxoma has extravasated red blood cells and thick-walled vessels, some of which may be large. Neither were seen in this lesion, but the smaller vessels with perivascular collagen deposition may be mistaken for thick-walled vessels of AA. The key histologic features for vulvar lymphedema are the dilated and tortuous lymphatics, which are not described in AA.3–6 The lesion Farshid and Weiss1 described as massive localized lymphedema has some overlap with our case, although differences exist. Their cases occurred in morbidly obese patients, most commonly in the thigh. Some of their cases histologically were worrisome for well-differentiated liposarcoma because of a fat component. Our case lacked fat, but was otherwise very similar histologically by the presence of clustered capillaries with perivascular inflammation, perineural edema, and ectatic lymphatics.
The present case should not be confused with the fibroepithelial polyp, which may have a histologic resemblance to our case. A fibroepithelial polyp is a better delineated, distinctly polypoid lesion that lacks dilated lymphatics and may contain atypical and multinucleated cells.7
Also in the differential diagnosis is superficial angiomyxoma.8 Although this lesion has much microscopic overlap with our case, superficial angiomyxoma has a characteristic nodular architecture, acellular myxoid clefts, and is grossly well-demarcated; none of these features was seen in our case. Superficial angiomyxoma lacks dilated and tortuous lymphatics.
Conventional types of neurofibroma can have myxoid changes. Wavy spindle cells, wirelike collagen bundles, Wagner-Meissner bodies, a clinical mass, and a lack of ectatic and tortuous lymphatics distinguish neurofibroma from our case. However, diffuse variants of neurofibroma may lack both the formation of a discrete mass and a wavy appearance of the stromal cells.9
The cause of lymphedema in this case is uncertain. As there was a history of repetitive repositioning of a urinary catheter, regional trauma resulting in lymphatic obstruction may be proposed as an etiology but cannot be proven. Because our patient had the unusual setting of being disabled (quadriplegic), this lesion could represent part of a rare clinicopathologic entity. Another case of labial (vulvar) hypertrophy was described in a patient who was also disabled (iron lung)10; the histopathology of that lesion was not reported.
In summary, a vulvar lesion that histologically resembles AA but lacks the characteristic thick-walled vessels and contains ectatic and tortuous lymphatics may represent hypertrophy with lymphedema. Awareness of this unusual condition in the vulva may prevent diagnostic errors.
JoviPak has some excellant compression garments as well
Acquired vulvar lymphangioma mimicking genital warts. A case report and review of the literature
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All About Lymphangiectasia
Support group for parents, patients, children who suffer from all forms of lymphangiectasia. This condition is caused by dilation of the lymphatics. It can affect the intestinal tract, lungs and other critical body areas.
Lymphatic Disorders Support Group @ Yahoo Groups
While we have a number of support groups for lymphedema… there is nothing out there for other lymphatic disorders. Because we have one of the most comprehensive information sites on all lymphatic disorders, I thought perhaps, it is time that one be offered.
Information and support for rare and unusual disorders affecting the lymph system. Includes lymphangiomas, lymphatic malformations, telangiectasia, hennekam's syndrome, distichiasis, Figueroa syndrome, ptosis syndrome, plus many more. Extensive database of information available through sister site Lymphedema People.
Teens with Lymphedema
All About Lymphoedema - Australia
Updated Jan. 12, 2011