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Nadine B. Skinner, MD New Hanover Regional Medical Vaginal pain and fever Center, Residency in Family Medicine, Wilmington, NC in a premenarchal girl How would you treat?

A 13-year-old girl is brought to your office by her father because she has had vaginal discomfort for 2 days. She also has had a fever up to 104˚F and a small perineal rash. She was seen the previous day in the emergency department (ED) for the same symptoms. The ED evaluation included urinalysis, urine pregnancy test, and complete blood count with differential. Results were reportedly normal, with the exception of a left shift without leukocytosis. Slightly indurated and “pebble-like” lesions were noted on the perineal exam. DNA probes for and were obtained. The patient was given acyclovir for presumed new-onset herpes simplex and was instructed to continue acetaminophen and ibuprofen for fever. The patient and her father are now seeking follow-up care. Q: What are some causes of vaginal pain with fever? How would you proceed with the evaluation? A:

Other medical history Physical examination • Enuresis, recurrent perioral rash • Review of systems: Positive for chills, around age 7 with annual recurrences fever, dysuria, and perineal pain; other- • No chronic infections or illness wise negative • Premenarchal • Temperature 101.6˚F, pulse 112, respi- • Negative trauma history, including ratory rate 22, blood pressure 110/70 abuse mm Hg, weight 94 lb • Bike riding 2 days earlier but no falls • Alert female in no distress, normal • Taking acyclovir as prescribed by ED; neurologic exam acetaminophen/ibuprofen for fever • HEENT: normal exam with clear FEATURE EDITORS • No known allergies oropharynx without lesions Audrey Paulman, MD, • Skin/integument: no rashes, including MMM, and Paul Paulman, Family and social history MD, University of Nebraska arms, legs, hands, feet, and trunk College of Medicine, Omaha • Parents alive and well; no siblings • Heart, lungs, and abdomen: tachy- • Straight-A student cardia, otherwise normal CORRESPONDING AUTHOR Nadine Skinner, MD, • Involved in JROTC (was on a retreat • Reproductive examination (chaperone Eastern North Carolina 2 weeks ago) present): Tanner stage III; perineal Medical Group, PLLC, 1041 • No use of tobacco, alcohol, street edema with marked purple ecchymoses Noell Lane, Suite 105, Rocky Mount, NC 27804. drugs, or suspicious substances located bilaterally at posterior E-mail: [email protected] • Denies any sexual activity introitus; multiple lacerations also

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noted as well as tenderness; some vaginal discharge; speculum and scabbed areas noted; no periurethral bimanual exam were deferred due to or perianal lesions or bruising; no pain.

Q: What is your presumptive diagnosis and plan? A:

You are concerned about abuse. You includes sexual abuse, herpes simplex interview the patient alone and she again virus, Behçet’s disease, Epstein-Barr infec- denies any type of sexual encounter. She tion, , Crohn’s disease, has never used tampons. She does report and . Definitive that she is a heavy sleeper and that she diagnosis can be difficult (TABLE 1). recently went on a retreat where she and One retrospective series reviewed the a girlfriend slept in the same room. As far case of 9 adolescent females with vulvar as she knows, there was no intruder. She ulcers and found that 6 had no formal tearfully says, “If anything happened, diagnosis. Most important, the initial I can’t remember.” presentation should prompt healthcare You also interview the father alone. He professionals to take steps to ensure a says he and his wife “keep a tight rein” on patient’s safety. their daughter. She has been out of their After performing a physical examina- supervision only for the recent retreat. He is tion, the abuse experts report that the concerned that his daughter may have been patient’s presentation is consistent with given a “date-rape” drug and requests test- Behçet’s disease. A genital culture was ing. His demeanor seems appropriate dur- obtained, and the patient was given pain FAST TRACK ing the conversations both in the presence medication and azithromycin. Initial presentation and absence of his daughter. The expert also stated that the father’s According to state law, you notify the reaction to the situation was appropriate of vulvar ulcers Department of Social Services of unex- and not that of an abusive father. Most should prompt plained perineal trauma. You also contact perpetrators are very hostile and defen- steps to ensure the local sexual abuse/rape experts in sive, whereas this father was extremely your area, who have arranged for an eval- concerned and cooperative. Still, a report a patient’s safety uation the following day. The father to Child Protective Services had to be assists in making the arrangements for the made because of the unexplained physical evaluation while he is still in your office. findings, especially in the genital area.

Further consideration ■ Department of Social Additional information on Behçet’s disease Services forensic interview is found in an article in the New England and medical examination Journal of Medicine from 1999: “Behçet’s The next day, the patient and her family disease is an inflammatory disorder of undergo a comprehensive evaluation unknown cause, characterized by recurrent by local medical and investigative profes- oral aphthous ulcers, genital ulcers, uveitis, sionals from the Department of Social and skin lesions.” Services. You ponder this information and note The for genital that your patient has no oral lesions ulcers in a sexually inexperienced female currently but apparently has a history of

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TABLE 1 • Rapid plasma reagin (RPR) nonreactive Differential diagnosis • Serum pregnancy negative of vulvar ulcers in sexually • Urine drug screen negative for PCP, inexperienced females benzodiazepines, amphetamines, THC, opiates, barbiturates, Sexual abuse methadone, tricyclic antidepressants • Complete blood count normal; white Herpes simplex virus infection blood count 9600 with a normal Behçet’s disease differential Epstein-Barr viral infection As the family physician, you interpret Crohn’s disease these results and support the clinical plan for this patient, pending further changes in Pilonidal disease the clinical picture. Hidradenitis suppurativa

some oral lesions. You review your records ■ Next contact— indicating a history of recurrent perioral ED visit 2 days later rash but do not find a history of oral The patient’s vaginal pain is worsening and lesions. However, the consultant remains she cannot void. Large ulcers with adhered constant in his diagnosis. pus are on the left labia; a smaller lesion is You review the results of the ED labo- on the right labia. There are also question- ratory evaluation: able lacerations of the posterior fourchette. • Gonorrhea/chlamydia DNA probe She is catheterized and 300 mL urine is negative emptied from the bladder. A gynecology • Herpes culture and herpes consultation is obtained by the ED physi- immunoglobulin M (IgM) negative cian with your concurrence.

Q: This patient has had an extensive work-up. Is there any other diagnostic FAST TRACK testing you would consider? In Behçet’s, oral A: ulcers appear in 70% of cases; genital lesions are often present Q: Is this Behçet’s disease? as well A:

■ Details of Behçet’s disease Clinical criteria of Behçet’s Behçet’s disease is most common in the Diagnosis of Behçet’s disease is based on third or fourth decade of life. It has an asso- clinical criteria. No single test can deter- ciation with the human leukocyte antigen mine if a patient has Behçet’s. HLA-B51 and HLA-B5 allele. Vascular An International Study Group of injuries, hyperfunction of neutrophils, and physicians was convened to develop a set autoimmune responses lead to the clinical of guidelines to diagnose Behçet’s disease. findings. Behçet’s disease is presumed to be These criteria include recurrent, painful an autoimmune disease, with the primary oral ulceration with at least 2 other lesion being related to the vasculitis. symptoms (recurrent genital ulcers,

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eye lesions, skin lesions, and positive TABLE 2 pathergy test) (TABLE 2). These diagnostic Diagnostic criteria criteria may be accessed at the website of Behçet’s disease of the American Behçet’s Disease Association, www.behcets.com. Recurrent oral ulceration • Aphthous ulcers or herpetiform ulcers Prevalence of signs • Three times in 12-month period

Oral ulcerations are the presenting signs AND AT LEAST 2 OTHER SYMPTOMS: in about 70% of cases. These ulcers may remain for as long as 3 weeks. In women, Recurrent genital ulcers the genital lesions are often present in the Eye lesions vulvo-vaginal region; in men, they often • Uveitis, vitreous cells on slit-lamp appear in the scrotal area. Ocular com- exam, retinal vasculitis plaints occur in about one-half of patients, sometimes with photophobia, watering, Skin lesions and blurred vision. The most common • Erythema nodosum, pseudofolliculitis, papulopustular lesions, or skin lesions are pseudofolliculitis and acneiform nodules erythema nodosum. The pathergy test uses a sterile needle Positive pathergy test to make a skin prick. The test result is pos- • Determined at 24 to 48 hours itive if an aseptic erythematous nodule or Adapted from Sakane et al, N Engl J Med 1999.1 pustule (>2 mm in diameter) occurs within 24 to 48 hours, indicating neutrophil hyperfunction. TABLE 3 Differential diagnosis Differential diagnosis of Behçet’s disease Skin, joint, gastrointestinal, vascular, and central nervous systems may also be Chronic oral aphthosis involved. The differential diagnosis includes FAST TRACK Herpes simplex virus infection chronic oral aphthosis, herpes simplex virus Treatment of infection, Sweet’s syndrome, ankylosing Sweet’s syndrome spondylitis, inflammatory bowel disease, Behçet’s depends and multiple sclerosis (TABLE 3). Ankylosing spondylitis on symptoms: oral and topical Laboratory findings and treatment Inflammatory bowel disease (Crohn’s disease, ulcerative colitis) Examination of the blood can reveal steroids, nonspecific findings consistent with Multiple sclerosis colchicine, topical inflammation, including elevated C- tetracycline, Adapted from Sakane et al, N Engl J Med 1999.1 reactive protein, erythrocyte sedimenta- anticoagulants, tion rate, and positive markers for autoimmune diseases. Colchicine may help alleviate oral and cytotoxic and Treatment depends upon the particular genital ulcers as well as skin, ocular, and antirheumatic symptoms and clinical findings and must joint problems. agents be coordinated with the various specialists Topical tetracycline may be used to involved. treat oral ulcers. Topical steroids are used for oral and Anticoagulants may be indicated for genital ulcers as well as ocular lesions. vascular disturbances and progressive cen- Oral steroids are useful for gastroin- tral nervous system lesions. testinal and neurological symptoms but Cytotoxic and antirheumatic agents may also help with skin, joint, vascular, may treat vascular, ocular, neurological, and ocular symptoms. and joint manifestations.

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Q: If the patient does not meet the criteria for Behçet’s disease, what are the next steps? A:

■ Gynecology consultation recommends changing the IV The gynecologist recommends that the from ampicillin/sulbactam to piperacillin/ patient be admitted to the hospital for tazobactam (Zosyn) with the addition of examination under anesthesia. This clindamycin for broad-spectrum cover- examination found an ulcerated area of age. Later, the final culture results from perineum, , and edema, without the sexual abuse evaluation show heavy a palpable . Necrotic tissue is Enterococcus colonization. Surgical cul- debrided. ture results reveal moderate Escherichia Biopsies are obtained for herpes, coli, moderate Enterococcus, light aerobic, and anaerobic cultures. Repeat Staphylococcus epidermidis, heavy bac- gonorrhea and chlamydia polymerase teroides, and moderate Lactobacillus. All chain reaction (PCR) probes are obtained are susceptible to the piperacillin/ as well. The patient is started on tazobactam. ampicillin/sulbactam (Unasyn) after the The patient does well and is successful- debridement. ly switched to oral amoxicillin/clavulanate Further laboratory testing is also (Augmentin) with no recurrent fever and done at admission, including a throat continued healing of vaginal ulcers. She is FAST TRACK culture, cryoglobulin, hepatitis screen, sent home to continue to heal. Further testing antinuclear antibody (ANA), HIV, lupus One week later, the patient is seen in anticoagulant, cardiolipin, herpes IgG, follow-up with continued healing and no includes biopsy antithrombin III level, immune complex further lesions. A few months later, the for herpes, PCR, detection panel for C1q, and tissue trans- patient has healed completely and no throat culture, gluttin AB4 IgA. All results are negative. other lesions have occurred. Incidental A possible rheumatology consultation is discussions included recent menarche. ANA, cardiolipin, also discussed. The family physician, patient, and and tests for Culture results from the previous patient’s family do a final review of all hepatitis, HIV, sexual abuse evaluation show strep and clinical and pathological findings. and lupus gram-negative rods. Initial cultures from The pathology report shows inflam- exam under anesthesia reveal gram- mation and necrosis. In addition, the vul- positive cocci, gram-positive rods, and var biopsy reveals “multiple hair follicles gram-negative rods on the Gram’s stain adjacent to the inflammation and necro- with final report pending. An infectious sis, raising the possibility of infected hair disease consultation is recommended. follicles and pilonidal disease.” The family physician agreed. Unlike the implications with the pre- sumed Behçet’s, the patient’s localized disease process has no long-term health ■ Infectious disease consequences. She is discharged with a consultation diagnosis of labial ulcers with cellulitis, The consultant believes streptococcal resolved urinary retention, and a patho- infection is the most likely culprit and logical diagnosis of pilonidal disease.

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Q: What considerations exist in the diagnosis of pilonidal disease? A:

■ Pilonidal disease from other disorders in the same region, Pilonidal disease, this patient’s final such as anal fistulas, perirectal , diagnosis, is more common than Behçet’s and hidradenitis suppurativa. disease. Men are affected more often Recurrences of pilonidal disease are than women, and ages range from puber- quite common, with painful inflamed ty to the third decade of life with masses, abscesses, or sinus tracts. An acute decreased frequency after the age of 45. abscess must be incised and drained. Hair Pilonidal disease becomes a problem removal by laser may prevent the growth when “nests of hair” become infected. It is of hair temporarily. For recurring infec- mostly found in the natal cleft but can tions, the definitive treatment is surgical. present in almost any hairy area, mostly in Procedures range from simple incision and the midline. Other examples include the curettage to excision of the area with plas- umbilicus and scalp. There are also reports tic flap reconstruction. are not of acquired pilonidal disease from trauma routinely needed, but they may be appro- in the hands of barbers, hair dressers, priate when cultures indicate specific sheep shearers, and animal groomers. It is microbes, when significant cellulitis is important to distinguish pilonidal disease present, or for perioperative prophylaxis.

Q: What are the comparative likelihoods of pilonidal disease and hidradenitis FAST TRACK suppurativa? Pilonidal disease A: can be found in the natal cleft, umbilicus, and scalp

Could it be hidradenitis suppurativa? This defect leads to trapping of bacteria and Hidradenitis suppurativa is a possibility, formation of firm, pea-sized nodules or but the clinical and pathological findings . Some associations between hidradeni- make pilonidal disease more likely. tis suppurativa and sex hormones have been Hidradenitis suppurativa similar to proposed, but hormonal states of pregnan- pilonidal disease, but it usually is located cy, menstruation, and menopause have not in the axillary, perineal, or inguinal areas. shown clear association with hidradenitis Incidence is as high as 1 in 300, and it fre- suppurativa. Initial symptoms of hidradeni- quently begins with puberty. tis suppurativa may be pruritus, erythema, Hidradenitis suppurativa differs from and hyperhidrosis. Recurrences are com- pilonidal disease in that the hair follicle mon. Treatment includes medications such becomes occluded due to a defect in the as antibiotics, antiandrogens, and retinoids, epithelium of the follicle’s terminal ends. as well as surgical excision. ■

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Family physician commentary

lthough this case is unusual, sev- dealing with such a sensitive situation. Aeral key points are worth noting. The physician cannot take away his or • The family physician as case coordi- her human side when presented with nator allows patients access to this kind of situation. The key is clear, specialized medical care, particularly unbiased communication with both if problems are potentially difficult the parent and the child. medically or socially. In this instance, The child can initially be inter- multispecialty care with family viewed with the parent present. Then physician support is what enabled the child should be interviewed alone the final diagnosis. in a nonthreatening environment. • The question remains: Why is a When the family physician takes the pre-menarchal female affected by time to sit and talk with the child, pilonidal disease? The most reason- the child may reveal things otherwise able explanation is that the patient not discussed. Any questions must began to experience other changes be age-appropriate and open-ended, associated with puberty including being careful not to lead the child. secondary hair growth. The “nests of The parent should also be interviewed hair” in the deep intergluteal regions alone in a nonconfrontational manner, may also have been stimulated, stating what is known at the present leading to the inflammation, necrosis, and what is the plan, including further and infection demonstrated by the evaluation and reports to appropriate pathology and culture findings. agencies. Again, a calm demeanor is • Fortunately, in follow up, the patient important. In all of these interviews, has done well and has continued to clear documentation is paramount. be followed by the family practitioner, Referral to child advocacy centers FAST TRACK who provided ongoing care and as this patient experienced can also When discussing interpretation for this family. be appropriate. Referral may allow the physician to serve as the case coordi- possible sexual Discussing sexual abuse. The nator as done in this instance. The abuse, talk to the approach to discussing possible physician can also remain neutral, parents alone sexual abuse is a difficult one. Many giving a unique perspective of the in a calm and physicians do not feel comfortable family as the situation is investigated. nonconfrontational manner ACKNOWLEDGEMENTS 3. Townsend CM, Beauchamp RD, Evers BM, Mattox K, eds. Sabiston Textbook of Surgery: The Biological The author would like to thank Jessie Junker, MD, Albert Basis of Modern Surgical Practice. 17th ed. Meyer, MD, and Barbara Walker, DO, at New Hanover Philadelphia, Pa: Saunders; 2004. Regional Medical Center, Residency in Family Medicine, Wilmington, NC; and Beth Deaton, FNP, at Wilmington 4. Stewart EG. Hidradenitis suppurativa. UpToDate [data- Health Access for Teens, Wilmington, NC. base]. Dated January 12, 2004. Waltham, Mass: UpToDate; 2004. 5. Deitch HR, Huppert J, Adams Hillard PJ. Unusual vul- CONFLICT OF INTEREST var ulcerations in young adolescent females. J Pediatr The author has no conflicts of interest to declare. Adolesc Gynecol 2004; 17:13–16. 6. Lisse JR. Behçet disease. Emedicine [website]. REFERENCES Updated October 18, 2004. Available at: http://www.emedicine.com/med/topic218.htm. 1. Sakane T, Takeno M, Suzuki N, Inaba G. Behçet’s dis- Accessed on September 23, 2005. ease. N Engl J Med 1999; 341:1284–1291. 7. American Behçet’s Disease Association website. 2. Sullivan DJ. Pilonidal disease. UpToDate [database]. Available at: www.behcets.com. Accessed on Dated October 28, 2003. Waltham, Mass: UpToDate; September 25, 2005. 2003.

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