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.
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).
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.
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.
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.
Conflicts of interest
There are no conflicts of interest.
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Keywords:© 2017 Egyptian Women's Dermatologic Society
genitalia; hidradenitis suppurativa; localized lymphedema