Secondary Logo

Journal Logo

Case report: lymphedema vulva secondary to hidradenitis suppurativa

Bains, Pooja; Mahajan, Ameesha

Journal of the Egyptian Women's Dermatologic Society: September 2017 - Volume 14 - Issue 3 - p 197–199
doi: 10.1097/01.EWX.0000511862.41153.2d
Case report
Free

Lymphedema of vulva is a rare entity characterized by chronic and progressive engorgement of tissues. It is a diagnostic challenge which is frequently untreated or treated at a late stage. The condition is highly debilitating because of the physical difficulty and mental agony associated with it. There are various underlying causes of lymphedema vulva but the association of lymphedema vulva and hidradenitis suppurativa (HS) is not well recognized. We would like to share the case report of a 35 year old female patient who developed vulval lymphoedema as a complication of longstanding HS.

Department of Dermatology, Shri Guru Ram Das Institute of Medical Sciences and Research, Amritsar, Punjab, India

Correspondence to Pooja Bains, MD, No. 89, B Block, Ranjit Avenue, Amritsar 143001, Punjab, India Tel: +20 183 2500121; e-mail: pjdhawan76@gmail.com

Received August 22, 2016

Accepted December 15, 2016

Back to Top | Article Outline

Case report

A 35-year-old obese, multiparous woman presented to the Skin Outpatient Department with chief complaint of a large, slowly progressing genital swelling of 13 years duration along with history of painful perineal and axillary draining sinuses since adolescence. The genital swelling was noticed 13 years back after her first vaginal delivery and has been slowly progressing in size. On examination, a large, multilobular, hypertrophic mass involving both labia majora, introitus, clitoris, and extending to pubic region was observed. The swelling was firm and nonpitting on palpation (Figs 1 and 2). On further examination, the gluteal cleft, bilateral inguinal region, and right axilla showed sinuses with linear bands studded with malodorous seropurulent discharge (Figs 3 and 4). The occurrence of these flexural lesions was traced back to her adolescence having a waxing and waning course. The inguinal, axillary, and cervical lymph nodes were not palpable. The deformed appearance of the vulva depressed her, but there was no difficulty in coitus. The patient was a housewife with two children. Her gynecological and obstetric history was uneventful with two normal vaginal deliveries. She had taken no specific treatment for her complaints, except for off and on course of oral antifungals and antibiotics. There was no history of any extramarital exposure. She was obese, healthy, and afebrile. There was no history of genital ulcer, lower-limb swelling, lower abdominal pain, and altered bowel habits. There was no history of chronic cough, fever, and weight loss in the patient. Family history of tuberculosis was negative. On investigation, her hemoglobin was 10.4 g%, total leukocyte count was 7800 cells/mm3, and erythrocyte sedimentation rate was 32 mm at the end of the first hour. Venereal disease research laboratory test of the patient and her husband was negative, and screening for HIV was negative. Mantoux test and sputum examination for acid-fast bacilli were negative. Her chest radiography, ultrasound abdomen, and pelvis did not reveal any discernible abnormalities. Peripheral blood film for microfilaria was negative. The PCR for Mycobacterium tuberculosis was also negative. Her skin biopsy was sent with the differential diagnosis of lymphedema secondary to hidradenitis suppurativa (HS), tuberculosis, or Crohn’s disease.

Figure 1

Figure 1

Figure 2

Figure 2

Figure 3

Figure 3

Figure 4

Figure 4

The skin biopsy report revealed marked thickening of papillary dermis with an increased number of thin collagen bundles separated by pale ground substance. The collagen bundles were arranged parallel to each other and perpendicular to the skin surface in the papillary dermis. There was an increased number of thick-walled capillaries and lymphatics in the papillary dermis, which had scattered lymphocytic infiltrate. No evidence of granulomatous inflammation was noticed in the biopsy specimen (Figs 5 and 6).

Figure 5

Figure 5

Figure 6

Figure 6

The patient was diagnosed with lymphedema secondary to HS and started on isotretinoin 30 mg along with a tapering course of prednisolone. On follow-up, there was a decrease in seropurulent discharge. She was advised excision and split thickness grafting for lymphedema, as this condition is unresponsive to medical treatment, but she refused.

Back to Top | Article Outline

Discussion

Lymphedema is a pathologic condition characterized by the regional accumulation of excessive amounts of interstitial protein-rich fluid. The underlying pathology is the imbalance between the demand for lymphatic flow and the capacity of the lymphatic circulation 1. This lymphatic stasis leads to proliferation of the hypodermal and dermal connective tissue and formation of a nonpitting, firm, redundant swelling due to the sclerosing fibrosis of subcutaneous tissue 2. The term ‘elephantiasis’ is given to the hyperkeratosis, verrucous, and condylomatous changes because of long-standing lymph stasis 3. The most common sites involved are legs, arms, and genitalia. Lymphedema can be either primary or secondary. The various causes of secondary lymphedema are filariasis, tuberculosis, malignancy, trauma, or iatrogenic causes. The iatrogenic causes are radiotherapy and radical mastectomy with surgical excision of draining lymph nodes 4. In a case series on localized lymphedema, women were more frequently affected by lymphedema than men 5. Other contributory factors are morbid obesity, underlying systemic diseases such as hypothyroidism and diabetes mellitus, extraintestinal manifestation of Crohn’s disease, chronic inflammatory disorders including HS, and bacterial cellulitis 5. Lymphedema of the vulva can be a postoperative complication after vulvectomy and inguino femoral lymphadenectomy 6. HS is a chronic, recurrent inflammatory disease affecting the cutaneous apocrine glands and resulting in their obstruction 7. Potential complications of HS include dermal contraction, local or systemic infection, and squamous cell carcinoma. The rare complications include disseminated infection, restricted limb mobility from scarring, rectal or urethral fistulas, and lymphedema caused by lymphatic injury from inflammation and scarring 8.

Lymphedema affects patients after severe and long-lasting HS. It is due to chronic inflammation that leads to blockade or destruction of local lymph drainage routes 9.The fact that not all patients with HS progress to lymphedema indicates that an underlying predisposition must exist that allows clinically apparent lymphedema to occur 5. We found only one case report of a 33-year-old African-American woman with a 15-year history of hidradenitis suppurative resulting in lymphedema 10. In our case, the prolonged and untreated HS along with obesity were the contributory factors resulting in lymph stasis and lymphedema. The more common causes of lymphedema vulva such as filariasis, lymphogranuloma venerum, and tuberculosis were ruled out because of lack of clinical and epidemiological evidence; peripheral blood film was negative for microfilaria; and PCR for M. tuberculosis was negative. The biopsy did not reveal any epitheloid or giant cell granulomas, ruling out Crohn’s disease.

Back to Top | Article Outline

Conclusion

Our case illustrates bilateral lymphedema of the vulva as a rare complication of HS. We believe that lymphedema is often undiagnosed or misdiagnosed, with many patients experiencing substantial delay until a correct diagnosis is established. These issues contribute to the high morbidity and disabling nature of the disease. We emphasize the need for proper history taking and thorough clinical examination, as it may help find the cause for the vulval lymphedema. HS, although rare, should be considered as a contributory factor in lymphedema vulva.

Back to Top | Article Outline

Conflicts of interest

There are no conflicts of interest.

Back to Top | Article Outline

References

1. Rockson SG. Lymphedema. Am J Med 2001; 110:288–295.
2. Ghassen T, Mehdi J, Khaireddine B, Tahar MA. Penoscrotal lymphedema secondary to hidradenitis suppurativa. J Case Rep 2015; 5:203–206.
3. Pandhi RK, Sood A. Valia RG, Valia AR. Disease of arteries, veins & lymphatics. IADVL textbook and atlas of dermatology. 2nd ed. Mumbai, Maharashtra, India: Bhalani Publishing House; 2001. 576–595.
4. Bradbury AW, Murie JA. Russel RCG, Williams NS, Bulstrode CJK. Lymphatic system. Bailey & Love’s short practice of surgery. 23rd ed. London, UK: Arnold; 2000. 250–269.
5. Lu S, Tran TA, Jones DM, Meyer DR, Ross JS, Fisher HA, Carlson JA. Localized lymphedema (elephantiasis): a case series and review of the literature. J Cutan Pathol 2009; 36:1–20.
6. Gaarenstroom KN, Kenter GG, Trimbos JB, Agous I, Amant F, Peters AA, Vergote I. Postoperative complications after vulvectomy and inguinofemoral lymphadenectomy using separate groin incisions. Int J Gynecol Cancer 2003; 13:522–527.
7. Hay RJ, Adrians BM. Burns T, Breathnach S, Cox N, Griffiths C. Bacterial infections. Rook’s textbook of dermatology, 8th ed. Oxford, UK: Wiley Blackwell; 2010. 30.1–30.82.
8. Shah N. Hidradenitis suppurativa: a treatment challenge. Am Fam Physician 2005; 72:1547–1552.
9. Faye O, Petit F, Poli F, Petit T, Wechsler J, Gabison G, et al. Lymphedema as a complication of hidradenitis suppurativa in three patients. Ann Dermatol Venereol 2007; 134:567–569.
10. Moosbrugger EA, Mutasim DF. Hidradenitis suppurative complicated by severe lymphedema and lymphangiectasias. J Am Acad Dermatol 2011; 64:1223–1224.
Keywords:

genitalia; hidradenitis suppurativa; localized lymphedema

© 2017 Egyptian Women's Dermatologic Society