Acquired vulvar lymphangioma: risk factors, disease associations, and management considerations: a systematic review : International Journal of Women's Dermatology

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Review Article

Acquired vulvar lymphangioma: risk factors, disease associations, and management considerations: a systematic review

Duong, Amber BSa; Balfour, Alex BSa; Kraus, Christina N. MDb,*

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International Journal of Women’s Dermatology 9(2):p e087, June 2023. | DOI: 10.1097/JW9.0000000000000087
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What is known about this subject with respect to women and their families?

  • Acquired vulvar lymphangioma (AVL) is a lymphatic neoplasm that is localized to the vulva, often appearing in the setting of prior pelvic malignancy or surgery.
  • Diagnosis is often delayed, and the condition is commonly refractory to treatment.

What is new from this article as messages for women and their families?

  • This systematic review found that AVL is commonly associated with prior pelvic malignancy and the subsequent surgical treatment, radiation, or lymph node dissection, and is seen in the setting of inflammatory bowel disease.
  • The most reported treatment modalities for AVL were excision, followed by laser therapy, but there were limited outcomes data and no information on quality-of-life following therapy.
  • In most cases, patients had been treated with other therapies without improvement, and the diagnosis was delayed.


Acquired vulvar lymphangioma (AVL), also known as acquired lymphatic anomaly or lymphangiectasia, and previously known as lymphangioma circumscriptum, is an uncommon condition characterized by an abnormality in the lymphatic vessels of the vulva.1,2 AVL is associated with obstructed or impaired pelvic lymph drainage,1 which can occur in the setting of chronic inflammatory or neoplastic conditions, as well as in the setting of prior surgical or radiation therapy that leads to lymphatic disruption. Studies have shown that AVL is often a late complication of anogenital and pelvic malignancies, with cervical carcinoma being the most common malignancy reported, but is also reported in inflammatory conditions including Crohn’s disease and infectious conditions such as tuberculosis.1,3,4

Clinically, AVL is often described as clusters of vesicular or verrucous papules,3 and superficial cases are sometimes described as resembling “frogspawn” with grouped clear to cloudy fluid-filled vesicles. Typically, vesicles have surrounding erythema and can appear as small confluent plaques (Fig. 1A). These features are often accompanied by surrounding edema (unilateral or bilateral) (Fig.1B) and even overlying eczematous change or lichenification. Symptoms may include pain, pruritus, discomfort, dyspareunia, and a burning sensation. In more superficial cases there may be fluid drainage and malodor.3,5–7 Information on the clinical course of AVL is limited, primarily due to few observational studies and the rarity of this condition.

Fig. 1.:
Clinical images of vulvar-acquired lymphatic anomaly and edema. (A) Erythematous to purple papulovesicles on right labium minus, representing acquired vulvar lymphatic anomaly. (B) Left-sided labium minus edema, representing vulvar lymphedema.

In early cases, AVL may have nonspecific clinical and histologic findings, often resulting in a misdiagnosis of inflammatory dermatitis. In cases where vesicles are more apparent, the differential includes herpes and autoimmune blistering disorders. When AVL presents as verrucous or vascular papules, neoplastic etiologies including condyloma acuminata, molluscum contagiosum, angiokeratomas, and even vascular lesions may be considered.3 Because of this variability in clinical findings, diagnosis is usually confirmed by tissue biopsy.4,8 Histologically, AVL typically presents with multiple dilated lymphatics in the superficial dermis. The epidermis may have overlying hyperkeratosis, acanthosis, and spongiosis.9

Studies on treatment are limited with no guidelines to date on the medical or surgical management of AVL. Various treatments have been reported including surgical excision, ablative and nonablative laser therapies, and topicals3,10,11 and all have been associated with a high rate of recurrence. The aim of this study is to perform a systematic review on cases of AVL to summarize the epidemiologic and clinical findings and identify associated comorbidities and treatment options.

Materials and methods

Literature search

This study was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (Fig. 2). A primary literature search was conducted using PubMed, CINAHL, and OVID, from all years to 2022. Two authors independently searched and cross-checked with the following search terms: (lymphangioma circumscriptum OR microcystic lymphatic malformation OR lymphangioma OR lymphangiectasia) AND (vulva OR vagina).

Fig. 2.:
Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRIMSA) flow diagram of literature search and article selection.

Article selection

Articles published in English from all years were considered for eligibility. Articles were excluded if they discussed congenital lymphangioma circumscriptum or nonvulvar presentations.

Data extraction

Included studies were summarized using a data extraction form with the following variables extracted: number of patients reported in the study, disease association, treatments for prior pelvic malignancy, patient ages, ethnicity, time between onset of AVL symptom onset and diagnosis, symptoms, clinical findings, treatment for AVL, duration/number of treatments, duration of follow-up, efficacy, reported recurrence, interval for follow-up, prior treatments, histopathology, status of quality of life measures, and inclusion of clinical or histopathological photos.


Literature review

The initial literature search yielded 290 articles. Overall, 146 were nonduplicate articles. A total of 54 articles were excluded based on their title and/or abstract, including 48 that were not related to AVL and 8 that were not available in the English language. No additional articles were identified based on a search of article references. Ultimately, 78 articles,1,3–79 comprising a total of 133 patients, met the eligibility criteria and were included in this qualitative analysis. Articles were published between 1979 and 2022. The eligible articles consisted of case series, case reports, literature reviews, and a retrospective chart review. Relevant variables extracted from each article are included in Table 1.

Table 1 - Variables extracted from studies included in literature review
PMID Type of study Number of patients Prior pelvic malignancy Other disease association Prior radiation Prior lymph node dissection Prior surgery or chemotherapy Patient age AVL duration before diagnosis Treatment type (T = topical therapy, PT = physical therapy, L = laser, S = surgical, or other) Treatment specifics (procedure, laser settings, medication) Interval for follow-up
1 34736616 Case report 1 None Crohn Disease None None None 56 3 years T Clobetasol 0.05% None
2 34877687 Review, case Series 6 n = 6 None n = 6 n = 5 None Median: 61,
Range: 44–72
median interval between malignancy diagnosis > acquired vulvar lymphangioma circumscriptum (AVLC) diagnosis: 10 years (0–32 years) S Excision (n = 5), Unknown (n = 1) 1.5 years, 3 years, 0.04 years, Unknown (n = 3)
3 14761137 Case report 1 None Pregnancy None None None 35 5 years None None None
4 2644602 Case report, review 1 None None None None None 42 None S Excision 6 weeks
5 8408931 Case report 1 None None None None None 46 None None None None
6 27329721 Case report 16 n = 9 None (n = 7) n = 7 n = 3 Hysterectomy (n = 4), Salpingo- oophorectomy (n = 3), Wide local excision (n = 1) Median: 55,
Range: 43–81
11.3 years between cancer treatment and vulvar lymphangioma circumscriptum (VLC) development (range: 5–20 years) Unknown Unknown None
7 15488131 Case report 1 None None None None None 17 4 S Excision 2 weeks
8 22529452 Case report 1 None Tuberculosis None None None 35 1 year S Excision (n = 2) None
9 8914364 Case report 1 None Recurrent cellulitis None None None 42 7 months T IV antibiotics and local antiseptic 5 years
10 7747544 Case report 1 None None None None None 76 2 years T None None
11 26967121 Literature review and case report 5 n = 2 Crohn’s Disease n = 1 None None Median: 50,
Range: 18–68
None L, S YAG Laser & Excision (n = 2), Electrodessication and curettage (n = 1), Excision (n = 1) None
12 23595194 Case report 1 None None None None None 24 since teenager Other Compression therapy None
13 25706522 Case report 1 n = 1 None n = 1 None Cystectomy (n = 1) 69 3 years None None None
14 11270296 Case report 1 None None None None None 44 6 None None None
15 21464721 Case series, review 1 None None None None None 39 None S Excision None
16 26374361 Case series, review 3 None None None None None Mean: 33.3, Standard Deviation: 8.4 None None None None
17 18312992 Case series, review 1 n = 1 None n = 1 n = 1 Hysterectomy (n = 1) 63 4 years S Excision 28 weeks
18 22615519 Case series 2 n = 2 None n = 2 None Hysterectomy (n = 2) 65, 44 10 years, 16 years S Excision Regular
19 30812056 Case report 1 None None None None None 61 1.5 years S Excision 4 weeks
20 16803506 Case series, retrospective study 1 n = 1 None n = 1 None None 40 6 months –2 years S Excision None
21 22199085 Case report (letter) 1 None None None None None 55 2 years S, L Hysterectomy, CO2 laser vaporization (defocused mode with a 2 mm spot size) 15 weeks
22 23130218 Case report (letter) 1 n = 1 None None n = 1 None 50 3 months None None None
23 2684843 Case series 3 n = 1 Crohn’s Disease (n = 2) n = 1 None Hysterectomy (n = 1) Median: 38,
Range: 37–38
2 years (n = 3) L, S, Other Excision, cryotherapy 2 months (n = 1)
24 8286236 Case report 2 n = 2 None n = 2 None Hysterectomy (n = 2) 49, 50 None S Excision 18 months
25 761961 Case report 1 None Tuberculosis (n = 1) None None Lymph node incision (n = 1) 62 30 years T, L Isonicotinic acid hydrazide, Ethambutolcholride, Rifampicin, Electocoagulation 6 months
26 30289772 Case report (letter) 1 n = 1 None n = 1 None Hysterectomy (n = 1) 78 10 years None None None
27 16219410 Case series, review 3 n = 2 Tuberculosis (n = 1) n = 1 n = 1 Complete surgical resection (n = 2), Hysterectomy (n = 2) Median: 67,
Range: 53–71
None L Laser therapy (n = 1); CO2 laser vaporization (n = 2) 4 months, 22 months, 90 months
28 21547888 Case report 2 n = 2 None n = 2 None Hysterectomy (n = 2) 56, 68 None S Excision 7 months, 20 months
29 24133609 Case report 1 None Crohn’s Disease None None None 35 Several years None None None
30 12218845 Case Report 2 n = 2 None n = 2 n = 2 Hysterectomy (n = 2) 75, 46 10 years L CO2 laser vaporization (n = 2) 3 months 8 months
31 28461089 Case series 1 None None None None None 68 None None None None
32 25099515 Case report (letter) 1 None Pregnancy (n = 1) None None None 28 1 month None None 2 months after delivery
33 22802470 Case report 1 None Breast Cancer (n = 1) None None Masectomy (n = 1), Chemotherapy (n = 1) 46 8 years S Excision (n = 2) 4 weeks post-op, 3 month return
34 1694423 Case report (letter) 1 n = 1 None n = 1 None Complete surgical resection (n = 1) 75 2 years L CO2 laser vaporization- multiple sessions. 18 months
35 4065708 Case report 1 n = 1 None None n = 1 Hysterectomy (n = 1) 51 None None None None
36 29451158 Case report (letter) 1 None Klippel-Trenaunay syndrome (n = 1) None None None 31 4 years None None None
37 10430005 Case report 1 n = 1 None n = 1 None Complete surgical resection (n = 1) 70 4 years L CO2 laser vaporization 4 weeks after wound healing: 5 more laser sessions
38 34621964 Case series 2 None Hiradenitis suppurativa (n = 2) None None None 44, 46 0 S Excision 3 years (n = 2)
39 15752314 Case report 1 None Tuberculosis (n = 1) None None None 22 5 years Other Oral isoniazid, rifampicin, pyearazinamide and ethambutol None
40 34837392 Case report 1 n = 1 None n = 1 None Hysterectomy (n = 1) 61 None L CO2 laser vaporization None
41 17684378 Case report 1 n = 1 None None n = 1 Hysterectomy (n = 1) 73 2 years S Lymphaticovenular Anastomosis (LVA) surgery 6 months
42 10235381 Case report 1 None Crohn’s Disease None None None 44 7 months Other Podophyllin None
43 1427807 Case report 1 None None None None None 20 2 years S Vulvectomy 9 months
44 28283172 Case report 1 None None None None None 47 None S None None
45 11270298 Case report 1 n = 1 n = 1 None None 79 1 year T Topical mid-potency corticosteroid None
46 15250899 Case report 1 None None None None None 20 10 years None None None
47 22901902 Case report 1 None None None None None 44 3 years T, Other Gentian Violet 1% solution, Cetrizine hydrochloride None
48 17656926 Case Report 1 None None None None Surgical resection (n = 1) 48 5 years S Excision 1 year
49 11776513 Case report, review 1 None None None None None 48 3 years S Excision 16 months
50 22361479 Case report 1 None None None None None 44 None T Antihistamines, emollients None
51 34149227 Case report 1 None None None None None 25 1 month S Excision None
52 26167062 Case report 1 None None None None None 60 4–5 years S Excision 1 year
53 10609498 Case report, review 1 n = 1 None n = 1 n = 1 Hysterectomy (n = 1), Salpingo- oophorectomy (n = 1) 65 10 years L, T, Other CO2 laser vaporization (multiple procedures), Silver sulfadiazine 6 weeks
54 19396719 Case series 8 n = 8 None n = 7 n = 7 Surgical excision (n = 7) Median: 48,
Range: 39-63
None S Excision (n = 5) None
55 34263326 Case report 1 n = 1 None n = 1 n = 1 Vulvectomy (n = 1), chemotherapy (n = 1) 70 3 years Other Sclerotherapy 4 weeks
56 22026919 Case report 1 n = 1 None n = 1 n = 1 Hysterectomy (n = 1) 42 4 years S, L Excision & CO2 laser vaporization (1st treatment), excision (2nd treatment) None
57 25468056 Case report 1 None None None None None 67 3 years None None None
58 15228436 Case report 2 n = 2 None n = 2 None None 30, 45 1 week, unknown (max 2 years) Other- penicillin Penicillin 6 months
59 28242996 Case report 1 None Tuberculosis (n = 1) None None None 25 None None None None
60 24396614 Case report 2 None None None None None 55, 60 25 years, unknown T Topical antihistamine None
61 27331134 Case report 1 n = 1 None n = 1 None Chemotherapy (n = 1) 55 3–4 years S Excision 5 months
62 12495108 Case report 1 None None None None None 48 None S Oral antibiotics followed by excision 2years
63 18397567 Case report 1 None Pregnancy None None None 30 4 months None None None
64 20004630 Case series 4 Cervical cancer (n = 1) Hodgkin lymphoma (n = 3) None None None Median: 44.5,
Range: 28–57
32.3 years, 10.6 years,
2.9 years, 0.2 years
S Prophylactic antibiotic therapy (amoxicillin clavulanic acid) and excision Median: 53 months
65 12738156 Case report, review 1 None None None None None 30 3 years S Excision None
66 367022 Case report 1 None None None None None 38 7 years S Excision None
67 25190008 Case report 1 n = 1 None n = 1 None Hysterectomy (n = 1) 76 6 years Other Cryotherapy 8 months
68 26156111 Retrospective chart review 8 n = 8 None n = 4 n = 8 None Median: 61.5,
Range: 36-77
None S Excision (n = 8) None
69 1765960 Case report 2 None None None None None 32, 35 1 year, 7 years S (n = 2), L (n = 1) Excision with laser vaporization, Excision 4 months
70 27502262 Case report 1 None None None None None 20 10 years None None None
71 1669286 Case report 1 None Tuberculosis (n = 1) None None None 40 3 months S Excision None
72 33423429 Case report 1 n = 1 None n = 1 n = 1 71 None None None None
73 31757874 Case report 1 None HSV (n = 1) None None None 43 6 years Other Lymphatic drainage, shave excision None
74 28791276 Case report 1 Rectal cancer (n = 1) None n = 1 None None 68 1year L, S Electrocautery and CO2 laser vaporization 6 months
75 20580481 Case report, letter 1 None None None None None 36 8 years S Excision 1 year
76 20300370 Case report 1 None None None None None 18 5 years L, Other Radiofrequency ablation, Sclerotherapy Monthly x3, 6 months, 2 years
77 18319007 Case report 1 None None None None None 45 8 years T, other Topical antihistamines and emollients, Cryotherapy None
78 6931304 Case report 1 n = 1 None n = 1 n = 1 None 54 22 years None None None

Clinical presentation and diagnosis

The age distribution ranged from 17 to 81 with a median of 49 years. There were 5 cases in pediatric patients that were not classified as congenital lymphangioma. Due to the lack of ethnicity data included, we could not determine any ethnic distribution for AVL. In studies that reported diagnostic delay, there was a mean delay of 5 years until the time of diagnosis. The most common symptoms reported included: clear oozing discharge, pain, and pruritus. Eleven patients were asymptomatic. The most common clinical findings included: multiple circumscribed papulovesicular lesions of varying sizes, often filled with clear fluid. Anatomical distribution was varied and involved the mons pubis, one or both labia majora, one or both labia minora, entire vulva, or entire vulva with spread to the thigh. All cases were biopsy-proven.

Treatment and outcomes

A total of 58 studies consisting of 96 patients evaluated treatment modalities. Of these, the most common treatment modality was surgery, followed by laser. Topical therapies included clobetasol cream and other topical corticosteroids, local antiseptics, isonicotinic acid hydrazide, ethambutol chloride, rifampicin, gentian violet 1% solution, emollients, silver sulfadiazine 1%, imiquimod and antihistamines. One study mentioned the use of physical therapy or lymphedema therapy. Follow-up was variable and not reported in most studies.

Of the 36 case reports that listed surgery as the treatment modality, 23 (64%) reported no evidence of recurrence for a total of 31 patients. Seven reports of 21 patients did not mention the efficacy of surgical excision, and all other reports noted some type of recurrence. There were 13 case reports which reported laser as a treatment modality. Of these, 6 reported a combination of topical or surgical treatment modalities in addition to laser therapy. Five reports of 6 patients (38%) had no evidence of recurrence. One report did not include treatment efficacy, and all other reports noted recurrence, most being minor or lesion size smaller than the primary lesions. The analysis also included 11 patients that were treated with topical therapies. Treatment outcomes were rarely reported, with some just noting symptom improvement. Most outcome measures relied on clinical improvement in signs and symptoms as evaluated by the provider. Quality-of-life outcome measures were not included in any of the studies.

Disease associations

Although 68% of cases had a disease association, the most common being a prior diagnosis of pelvic malignancy (cervical, vulvar, vaginal, endometrial, or bladder). Inflammatory bowel disease (IBD) was the second most common disease association and was seen in 5% of cases. There were 3 cases (2%) presenting with lesions associated with pregnancy. Most patients had prior radiation or surgical treatment with 36% (n = 48) treated previously with radiation, 30% (n = 40) with prior lymph node dissection, and 27% (n = 36) with prior surgical resection without lymph node dissection. While the other 32% of cases were idiopathic, many did evaluate for other disease associations or malignancies with a variety of modalities. The most used diagnostic tools were screens for sexually transmitted infections such as syphilis, human immunodeficiency virus, or hepatitis. Other tests included ultrasound, abdominal and pelvic computed tomography or magnetic resonance imaging, and pap smears.


In this review, we summarize the clinical findings, treatment modalities, and disease associations of AVL. This is an underrecognized condition and diagnosis is often delayed with 84% of cases in our study reporting diagnostic delay. Prior studies have evaluated the association between AVL and malignancy and reported that AVL is a late complication of prior surgery or chemotherapy to treat anogenital and pelvic malignancies, with the most common preceding malignancy being cervical carcinoma.3 AVL is likely more common in pelvic and anogenital cancer survivors than reported as it is often misdiagnosed. Because it can present initially with nonspecific inflammation and symptoms of pruritus/burning, it may be misdiagnosed as other vulvar conditions including the genitourinary syndrome of menopause, lichen sclerosus, and contact dermatitis.

AVL is thought to develop due to disruption in lymphatic drainage, through surgical resection, lymph node dissection, radiation, or even mass effect of anogenital/pelvic malignancies.3 Because of the changes in drainage of lymphatic fluid, many cases of AVL have unilateral or bilateral vulvar edema. Vulvar edema alone may be an early presenting sign. Other conditions that lead to systemic inflammation, including IBD, specifically Crohn’s disease, have been associated with AVL. The mechanism is likely through long-standing edema, fibrosis, and inflammatory changes. Both vulvar edema and AVL are nonspecific findings that can be a presenting sign in Crohn’s disease or occur even in the setting of Crohn’s disease where the gastrointestinal disease is well-controlled or not symptomatic.1,10,29,34

Overall, a thorough examination, history, and high clinical suspicion is required to diagnose these conditions early. We recommend that in patients presenting with AVL with no prior diagnosis of IBD or malignancy, a thorough review of systems and age-appropriate screening should be performed. If there is a concern for pelvic malignancy, imaging, and appropriate lab work should be considered in the appropriate patient. If there is suspicion of IBD, appropriate workup including a fecal calprotectin level, may be considered.

Evidence-based guidelines for the management of AVL are lacking, leaving no clear standards for treatment. Surgical excision was found to have the lowest rate of recurrence when compared to laser and topical treatments, however, follow-up duration and reported outcomes were not standardized, making the evaluation of therapy efficacy challenging. Additionally, it is important to note that wide local excisions/labiectomy/vulvectomy may carry increased morbidity and functional impairment compared to nonsurgical methods. Overall, information regarding the duration of therapy, treatment time to resolution or recurrence, and efficacy measures were limited and variable.

Treatment requires multidisciplinary care and should focus on addressing any underlying lymphatic changes, managing any underlying inflammatory condition, and utilization of skin-directed therapies and barrier agents, as well as addressing symptoms of pruritus and pain. While only mentioned in one of the articles click or tap here to enter text., these authors suggest in addition to the above, referral to a physical therapist with specialized training in the management of genital edema.19

The main limitation of this review is the study design as data was derived predominantly from case reports and case studies and thus, our study is limited by interstudy variability and heterogeneity of results. Many studies were missing variables that are useful for providing diagnostic or therapeutic recommendations.

Summarizing the data from existing studies provides further information on the characteristics and treatment modalities of AVL. However, prospective studies are needed to further characterize this condition to better understand true incidence and prevalence, risk factors, and pathogenesis, with the goal of earlier diagnosis and development of treatment guidelines. Additionally, because symptoms can be debilitating, future studies should consider incorporating quality-of-life outcome measures.

Conflicts of interest




Study approval


Author contributions

AD, AB, and CK participated in screening articles, writing the manuscript, and editing the draft.


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lymphangiectasia; lymphangioma; lymphatic anomaly; vulvar edema

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