Lymphangioma circumscriptum as an untoward consequence of hidradenitis suppurativa surgery

Dear Editors,

Vulvar lymphangioma circumscriptum (LC) is a disorder of the lymphatics that can be congenital or acquired and cause significant physical and emotional discomfort. We present two cases of vulvar LC after surgical excision of inguinal sinus tracts for severe hidradenitis suppurativa (HS).

Case one was a 44-year-old, obese, female nonsmoker presented with new mildly painful lesions on the labia majora. She had a history of severe HS with her first and only surgical excision of inguinal sinus tracts 7 years prior to presentation. Prior treatments for these lesions included cryotherapy and silver nitrate without resolution. On examination, multiple clusters of pink–clear papules were noted on the vaginal introitus and left labia majora, and some papules had an overlying crust. Pathology demonstrated thin-walled vascular structures within the papillary dermis, surrounded by a collarette of epidermis and D2-40-positive endothelial cell staining, consistent with lymphangioma (Fig. 1).

Fig. 1.

Clusters of clear-to-pink papules on the vaginal introitus.

Case two was a 46-year-old, obese, female nonsmoker presented to the dermatology clinic for newly developed irritating lesions on her labia majora 5 years after her first and only surgical excision of inguinal sinus tracts for severe HS. She had no prior treatments, and examination showed clustered pink-skin-colored pseudovesicles on the bilateral labia. Pathology was consistent with lymphangioma (Fig. 2).

Fig. 2.

Bilateral labia with pink–tan, thin pseudovesicles.

Both patients underwent surgical excision of lymphangiomas without clinical recurrence at 3 and 3 years of follow-up, respectively.

LC is a benign entity that can be of primary or secondary origin. Primary lesions are present at birth or develop in the first few years of childhood. Secondary vulvar LC is reported as a consequence of radiotherapy, malignancies (the most common being pelvic carcinomas), fistulous Crohn disease, chronic scrofuloderma in tuberculous-positive patients, surgical manipulation including tumor removal or HS sinus tract removal, and chronic lymphedema (Chang et al., 2016, Jappe et al., 2002, Weyers et al., 1990).

The pathogenesis of the development of these lesions includes disruption of the lymphatics from a tumor, prior surgical procedure, or chronic lymphedema, resulting in large multilobulated and dilated cisterns that extend into the dermis with variability of depth. Evidence has shown that these cisterns do not communicate with adjacent lymphatics (Patel and Schwartz, 2009).

Discussion regarding treatment of LC should be done with every patient because this condition can cause significant physical and emotional distress. Lesions can initially be misdiagnosed as herpes virus outbreaks or condyloma acuminata, which can affect the emotional psyche of a patient due to the negative connotation of such diagnoses (Chang et al., 2016). Physical symptoms, including pruritus, exudative discharge, secondary infection, and edema, can also be a reason for treatment (Ghaemmaghami et al., 2008, Vlastos et al., 2003).

Treatments for secondary LC include observation, topical sirolimus, surgical wide local excision, electrodessication and curettage, ablative laser therapy, and referral to lymphedema clinic for maintenance of chronic lymphedema (Çalışkan et al., 2018, Chang et al., 2016, Huilgol et al., 2002, Jappe et al., 2002, Patel and Schwartz, 2009, Piernick et al., 2018, Sims et al., 2010, Weyers et al., 1990). Chang et al. (2016) reviewed 11 cases of acquired lymphangiectasia of the vulva and found that the most common treatment chosen was observation, followed by surgical excision. Rare cases of squamous cell carcinoma arising in vulvar LC have been reported, and patients should be followed clinically if resection is not performed (Short and Peacock, 1995).

Surgical removal of sinus tracts in severe refractory HS is limited to patients who have failed medical therapy. However, excision can lead to the unwanted side effect of LC, as demonstrated in the two cases presented here. Optimizing medical management for HS may avoid the negative physical and mental effects that LC of the vulva can have on patients. Medical treatments include topical therapy, such as soaps and antibiotics, and oral therapy, including oral antibiotic regimens. Newer therapies approved by the U.S. Food and Drug Administration include tumor necrosis factor alpha inhibitors, interleukin-23 inhibitors, and radiation therapy (Jemec, 2012).

It is recommended that all medical treatment options be used because surgery to remove sinus tracts deep in the dermis can lead to LC in similar deep dermal layers, making treatment of the lesions more difficult (Patel and Schwartz, 2009, Piernick et al., 2018). If surgical removal is indicated in patients with severe HS, follow-up for lesions is recommended to monitor for postoperative growth of LC. Lapolla et al. (1985) reported a case of LC of the vulva 10 years after radical hysterectomy and pelvic lymphadenectomy; notably, the patient developed lymphedema 4 years prior to presenting with LC. Similarly, Piernick et al. (2018) reported a case of scrotal LC 5 years after sinus tract removal for refractory HS. The cases presented in this report LC of the vulva appeared between 5 and 7 years after sinus tract removal. Both patients chose surgical excision of vulvar LC and did not have recurrence at follow-up between 3 and 5 years.

Severe HS can be a debilitating disease, and surgical removal of sinus tracts is often the last therapeutic option. For patients who have undergone sinus tract excision, the authors recommend yearly follow-up monitoring for the development of vulvar LC and a discussion with patients about treatment options if LC is symptomatic. Further studies are needed to discern the interval between sinus tract removal and LC development, as well as LC recurrence after surgical removal.

Conflicts of interest

None.

Funding

None.

Study approval

The author(s) confirm that any aspect of the work covered in this manuscript that has involved human patients has been conducted with the ethical approval of all relevant bodies.