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Vulvar edema in a pregnant adolescent
Adolesc Pediatr Gynecol (1990) 3:210-211
Adolescent
and Pediatric
Gynecology
.i: 1990 Springer-Verlag New York Inc.
Vulvar Edema in a Pregnant Adolescent N_ Thad Padua, M.D. and Samuel K. Parrish, M.D. Division of Adolescent Medicine. Department of Pediatrics. Medical College of Pennsylvania. Philadelphia. Pennsylvania
Abstract. We report a case of a pregnant adolescent female with vulvar edema as the initial presentation of nephrotic syndrome. Though adolescents with symptoms related to the genitalia are often presumed to have a sexually transmitted disease. this case illustrates that other conditions should also be considered. The differential diagnosis of vulvar edema is discussed.
Key Words. Nephrotic syndrome- Vulvar edemaAdolescent pregnancy Case Report A l6-year-old black girl presented complaining of a "lump in the private area." She acknowledged sexual activity without any contraception but denied vaginal discharge or bleeding. dysuria, fever, chilts. or abdominal pain. Her last menses occurred 1 month prior to the visit and was normal. She denied any history of sexually transmitted disease. Examination of the external genitalia revealed nontender edema and erythema of the left labia majora and minora without evidence of a discrete mass. The overlying skin appeared excoriated with erythema along the medial aspects of the labia. The abdominal exam revealed an obese girl; there was no tenderness or organomegaly. The uterus was not palpable. The remainder of the physical examination was normal for age. A diagnosis of early Bartholin's gland inflammation was made on the basis of unilateral presentation and erythema. The patient was instructed to take warm sitz baths and return in 2 days. She returned the next day because of increased vulvar edema and discomfort on walking. Reexamination of the genitalia revealed marked asymmetric Address reprint requests 10: N. Thad Padua. M.D .• Division of Adolescent Medicine. Department of Pediatrics. Medical College of Pennsylvania. 3300 Henry Avenlle, Philadelphia. PA 19129, USA.
swelling of the labia with the left side greater than the right. The edema was so extensive that the patient could not bring her legs together. Pelvic examination revealed an 18-week size uterus. Ultrasound was done to rule out a pelvic mass and the uterus was consistent with a size of 18 weeks. A diagnosis of Bartholin's abscess was still considered on the basis of erythema, asymmetry. and discomfort and she was recommended for admission for parenteral antibiotics. Another consultant noted puffiness of the patient's upper eyelids and pitting edema in both ankles. A urine dipstick revealed 4 + proteinuria and a urine pregnancy test was positive. Initial laboratory results included urinalysis specific gravity 1.038, pH 6.0. 4+ protein. 2 + ketones, 1 + blood, 20-30 white blood cells (WBCs), 1-4 red blood cells (RBCs), 2-5 hyaline casts; serum blood urea nitrogen (BUN) was 9.0 mg/dL creatinine 0.5 mg/dL total protein 3.5 gm/dl. albumin 1.2 g/dl, and cholesterol 466 mg/d/; hemoglobin was [2.1 giL, WBC 9100 Imm" with 69% segmented cells, 8% band forms, 17% lymphocytes, and 6% mononuclear cells. A 24hour urine collection had 13.5 g of protein. Other laboratory studies included rheumatoid factor, rapid plasma reagin, antinuclear antibodies, and antistreptolysin-O titer, all of which were negative. Cervical cultures for gonorrhea. chlamydia, and herpes were negative. These findings were diagnostic of nephrotic syndrome and treatment with oral prednisone, 2 mg/kg daily. was initiated. The patient's edema decreased gradually during her [-month hospital stay. Proteinuria and hypoproteinemia persisted during steroid treatment. The hospital course was complicated by pulmonary edema. treated with Lasix, and steroid-induced diabetes, which required insulin treatment. She was discharged on fluid and sodium restrictions and a tapering dose of prednisone. Four months later she delivered a healthy, term. male infant. A renal biopsy was done the following month and showed that the patient had focal sclerosing glomerulonephritis.
Padua & Parrish:
Vulvar Edema in Pregnancy
Discussion Vulvar edema can be the initial manifestation of a number of disorders and is a recognized feature of childhood nephrotic syndrome. The edema of nephrosis is usually first noted in the periorbital region and lower extremities but can accumulate in dependent sites such as the perineum. I Abdominal pain , associated with anorexia and occasional diarrhea , is also common in nephrotic syndrome. Crohn 's disease is another systemic disorder that can present with vulvar edema. A few cases have been reported with vulvar regions as the sole presenting symptom of the disease. " This extraintestinal manifestation of Crohn's disease may precede intestinal involvement by many years. Probably the most common cause of vulvar edema is infectious, usually related to sexually transmitted diseases . I nfectious entities include herpetic vulvitis which is often painful, with ulcerative and excoriated lesions. Associated systemic manifestations include fever. malaise, and fatigue. Bartholin's gland or duct abscess due to gonorrhea or chlamydia is also common. Vulvar swelling is often unilateral with excoriation and tenderness. Lymphogranuloma venereum due to chlamydial infection can cause vulvar swelling when lymph nodes are enlarged with bubo formation . This infection is often associated with sinus lesions. Chancroid due to Hemophilus ducreyi may also cause vulvar swelling associated with bubo formation and ulcerative lesions. Other less common causes of vulvar edema include vulvar hematomas which may be due to common trauma such as straddle injuries, rape, or sexual abuse. However, hematomas do not appear as simple vulvar edema. Vulvar hernias, more common in infants, may occasionally be caused by pudendal or vulvar extension of a congenital inguinal hernia. Inflammatory or parasitic blockage of vulvar lymphatics may cause elephantiasis. Sebaceous or inclusion cysts of the inner labia majora or minora arc usually small and asymptomatic but may enlarge and present as vulvar swelling. A cyst of the Canal of Nuck is a mass formed by separation of peritoneum attached to the round ligament as it inserts into the labia majora. Fluid may
211
accumulate in this peritoneal remnant and form a cystic dilatation. Vulvar edema may also result from intrapelvic pressure due to large tumors, marked ascites, or metastatic tumor in regional lymph nodes. Pregnancy, as well, can cause secondary lymphostasis due to pressure phenomenon. Benign neoplasms such as lipoma, fibroma , or hidradenoma may present as labial masses that can be mistaken for inflammatory processes . Reported benign tumors in childhood include granular cell myoblastoma, teratoma, and lymphangioma.' Though malignant neoplasms are more common in women older than 60 years, vulvar neoplasms such as squamous cell carcinoma, adenocarcinoma, and sarcoma botryoides have been reported in children.~ Lastly, dermatologic conditions such as contact dermatitis or allergic dermatitis can present with vulvar edema." Both present acutely on the vulva as edematous, erythematous , oozing lesions that may be accompanied by vesicles or pustules. If chronic , the skin may appear thickened and lichenified. The adolescent with vulvar edema is most likely to have a sexually transmitted disease, but the differential diagnosis of vulvar edema is extensive. Less common diseases with potential for significant medical complications such as nephrotic syndrome, should still be considered . Physicians caring for adolescents should avoid the bias of a narrow focus on sexually transmitted disease as an explanation of the findings in their patients. References I. Rudolph A, Hoffman J: Pediatrics. Norwalk, Connecticut, Appleton & Lange , 19~7, pp 1176-1177
2. Kremer M, Nussenson E. Steinfeld M, Zuckerman P: Crohn"s disease of the vulva. Am J Gastroenterol 19~4: 79:376 3. Huffman J, Dewhurst C. Capraro V: The Gynecology of Childhood and Adolescence , 2nd ed . Philadelphia, WB Saunders, 19~L pp 226 - 233 4. LaVecchia C. Draper GJ, Franceschi S: Childhood nonovarian female genital tract cancers in Britain 19621978. Cancer 19~4: 5: 188 5. Altchek A: Vulvovaginitis. vulvar skin disease, and pelvic inflammatory disease. Pediatr Clin North Am 19~ I: 28:416
Adolescent
and Pediatric
Gynecology
.i: 1990 Springer-Verlag New York Inc.
Vulvar Edema in a Pregnant Adolescent N_ Thad Padua, M.D. and Samuel K. Parrish, M.D. Division of Adolescent Medicine. Department of Pediatrics. Medical College of Pennsylvania. Philadelphia. Pennsylvania
Abstract. We report a case of a pregnant adolescent female with vulvar edema as the initial presentation of nephrotic syndrome. Though adolescents with symptoms related to the genitalia are often presumed to have a sexually transmitted disease. this case illustrates that other conditions should also be considered. The differential diagnosis of vulvar edema is discussed.
Key Words. Nephrotic syndrome- Vulvar edemaAdolescent pregnancy Case Report A l6-year-old black girl presented complaining of a "lump in the private area." She acknowledged sexual activity without any contraception but denied vaginal discharge or bleeding. dysuria, fever, chilts. or abdominal pain. Her last menses occurred 1 month prior to the visit and was normal. She denied any history of sexually transmitted disease. Examination of the external genitalia revealed nontender edema and erythema of the left labia majora and minora without evidence of a discrete mass. The overlying skin appeared excoriated with erythema along the medial aspects of the labia. The abdominal exam revealed an obese girl; there was no tenderness or organomegaly. The uterus was not palpable. The remainder of the physical examination was normal for age. A diagnosis of early Bartholin's gland inflammation was made on the basis of unilateral presentation and erythema. The patient was instructed to take warm sitz baths and return in 2 days. She returned the next day because of increased vulvar edema and discomfort on walking. Reexamination of the genitalia revealed marked asymmetric Address reprint requests 10: N. Thad Padua. M.D .• Division of Adolescent Medicine. Department of Pediatrics. Medical College of Pennsylvania. 3300 Henry Avenlle, Philadelphia. PA 19129, USA.
swelling of the labia with the left side greater than the right. The edema was so extensive that the patient could not bring her legs together. Pelvic examination revealed an 18-week size uterus. Ultrasound was done to rule out a pelvic mass and the uterus was consistent with a size of 18 weeks. A diagnosis of Bartholin's abscess was still considered on the basis of erythema, asymmetry. and discomfort and she was recommended for admission for parenteral antibiotics. Another consultant noted puffiness of the patient's upper eyelids and pitting edema in both ankles. A urine dipstick revealed 4 + proteinuria and a urine pregnancy test was positive. Initial laboratory results included urinalysis specific gravity 1.038, pH 6.0. 4+ protein. 2 + ketones, 1 + blood, 20-30 white blood cells (WBCs), 1-4 red blood cells (RBCs), 2-5 hyaline casts; serum blood urea nitrogen (BUN) was 9.0 mg/dL creatinine 0.5 mg/dL total protein 3.5 gm/dl. albumin 1.2 g/dl, and cholesterol 466 mg/d/; hemoglobin was [2.1 giL, WBC 9100 Imm" with 69% segmented cells, 8% band forms, 17% lymphocytes, and 6% mononuclear cells. A 24hour urine collection had 13.5 g of protein. Other laboratory studies included rheumatoid factor, rapid plasma reagin, antinuclear antibodies, and antistreptolysin-O titer, all of which were negative. Cervical cultures for gonorrhea. chlamydia, and herpes were negative. These findings were diagnostic of nephrotic syndrome and treatment with oral prednisone, 2 mg/kg daily. was initiated. The patient's edema decreased gradually during her [-month hospital stay. Proteinuria and hypoproteinemia persisted during steroid treatment. The hospital course was complicated by pulmonary edema. treated with Lasix, and steroid-induced diabetes, which required insulin treatment. She was discharged on fluid and sodium restrictions and a tapering dose of prednisone. Four months later she delivered a healthy, term. male infant. A renal biopsy was done the following month and showed that the patient had focal sclerosing glomerulonephritis.
Padua & Parrish:
Vulvar Edema in Pregnancy
Discussion Vulvar edema can be the initial manifestation of a number of disorders and is a recognized feature of childhood nephrotic syndrome. The edema of nephrosis is usually first noted in the periorbital region and lower extremities but can accumulate in dependent sites such as the perineum. I Abdominal pain , associated with anorexia and occasional diarrhea , is also common in nephrotic syndrome. Crohn 's disease is another systemic disorder that can present with vulvar edema. A few cases have been reported with vulvar regions as the sole presenting symptom of the disease. " This extraintestinal manifestation of Crohn's disease may precede intestinal involvement by many years. Probably the most common cause of vulvar edema is infectious, usually related to sexually transmitted diseases . I nfectious entities include herpetic vulvitis which is often painful, with ulcerative and excoriated lesions. Associated systemic manifestations include fever. malaise, and fatigue. Bartholin's gland or duct abscess due to gonorrhea or chlamydia is also common. Vulvar swelling is often unilateral with excoriation and tenderness. Lymphogranuloma venereum due to chlamydial infection can cause vulvar swelling when lymph nodes are enlarged with bubo formation . This infection is often associated with sinus lesions. Chancroid due to Hemophilus ducreyi may also cause vulvar swelling associated with bubo formation and ulcerative lesions. Other less common causes of vulvar edema include vulvar hematomas which may be due to common trauma such as straddle injuries, rape, or sexual abuse. However, hematomas do not appear as simple vulvar edema. Vulvar hernias, more common in infants, may occasionally be caused by pudendal or vulvar extension of a congenital inguinal hernia. Inflammatory or parasitic blockage of vulvar lymphatics may cause elephantiasis. Sebaceous or inclusion cysts of the inner labia majora or minora arc usually small and asymptomatic but may enlarge and present as vulvar swelling. A cyst of the Canal of Nuck is a mass formed by separation of peritoneum attached to the round ligament as it inserts into the labia majora. Fluid may
211
accumulate in this peritoneal remnant and form a cystic dilatation. Vulvar edema may also result from intrapelvic pressure due to large tumors, marked ascites, or metastatic tumor in regional lymph nodes. Pregnancy, as well, can cause secondary lymphostasis due to pressure phenomenon. Benign neoplasms such as lipoma, fibroma , or hidradenoma may present as labial masses that can be mistaken for inflammatory processes . Reported benign tumors in childhood include granular cell myoblastoma, teratoma, and lymphangioma.' Though malignant neoplasms are more common in women older than 60 years, vulvar neoplasms such as squamous cell carcinoma, adenocarcinoma, and sarcoma botryoides have been reported in children.~ Lastly, dermatologic conditions such as contact dermatitis or allergic dermatitis can present with vulvar edema." Both present acutely on the vulva as edematous, erythematous , oozing lesions that may be accompanied by vesicles or pustules. If chronic , the skin may appear thickened and lichenified. The adolescent with vulvar edema is most likely to have a sexually transmitted disease, but the differential diagnosis of vulvar edema is extensive. Less common diseases with potential for significant medical complications such as nephrotic syndrome, should still be considered . Physicians caring for adolescents should avoid the bias of a narrow focus on sexually transmitted disease as an explanation of the findings in their patients. References I. Rudolph A, Hoffman J: Pediatrics. Norwalk, Connecticut, Appleton & Lange , 19~7, pp 1176-1177
2. Kremer M, Nussenson E. Steinfeld M, Zuckerman P: Crohn"s disease of the vulva. Am J Gastroenterol 19~4: 79:376 3. Huffman J, Dewhurst C. Capraro V: The Gynecology of Childhood and Adolescence , 2nd ed . Philadelphia, WB Saunders, 19~L pp 226 - 233 4. LaVecchia C. Draper GJ, Franceschi S: Childhood nonovarian female genital tract cancers in Britain 19621978. Cancer 19~4: 5: 188 5. Altchek A: Vulvovaginitis. vulvar skin disease, and pelvic inflammatory disease. Pediatr Clin North Am 19~ I: 28:416