TABLE 3
Differential diagnosis of Behçet’s disease
| Chronic oral aphthosis |
| Herpes simplex virus infection |
| Sweet’s syndrome |
| Ankylosing spondylitis |
| Inflammatory bowel disease (Crohn’s disease, ulcerative colitis) |
| Multiple sclerosis |
| Adapted from Sakane et al, N Engl J Med 1999.1 |
Gynecology consultation
The gynecologist recommends that the patient be admitted to the hospital for examination under anesthesia. This examination found an ulcerated area of perineum, cellulitis, and edema, without a palpable abscess. Necrotic tissue is debrided.
Biopsies are obtained for herpes, aerobic, and anaerobic cultures. Repeat gonorrhea and chlamydia polymerase chain reaction (PCR) probes are obtained as well. The patient is started on ampicillin/sulbactam (Unasyn) after the debridement.
Further laboratory testing is also done at admission, including a throat culture, cryoglobulin, hepatitis screen, antinuclear antibody (ANA), HIV, lupus anticoagulant, cardiolipin, herpes IgG, antithrombin III level, immune complex detection panel for C1q, and tissue transgluttin AB4 IgA. All results are negative. A possible rheumatology consultation is also discussed.
Culture results from the previous sexual abuse evaluation show strep and gramnegative rods. Initial cultures from exam under anesthesia reveal grampositive cocci, grampositive rods, and gramnegative rods on the Gram’s stain with final report pending. An infectious disease consultation is recommended. The family physician agreed.
Infectious disease consultation
The consultant believes streptococcal infection is the most likely culprit and recommends changing the IV antibiotic from ampicillin/sulbactam to piperacillin/tazobactam (Zosyn) with the addition of clindamycin for broad-spectrum coverage. Later, the final culture results from the sexual abuse evaluation show heavy Enterococcus colonization. Surgical culture results reveal moderate Escherichia coli, moderate Enterococcus, light Staphylococcus epidermidis, heavy bacteroides, and moderate Lactobacillus. All are susceptible to the piperacillin/tazobactam.
The patient does well and is successfully switched to oral amoxicillin/clavulanate (Augmentin) with no recurrent fever and continued healing of vaginal ulcers. She is sent home to continue to heal.
One week later, the patient is seen in follow-up with continued healing and no further lesions. A few months later, the patient has healed completely and no other lesions have occurred. Incidental discussions included recent menarche. The family physician, patient, and patient’s family do a final review of all clinical and pathological findings.
The pathology report shows inflammation and necrosis. In addition, the vulvar biopsy reveals “multiple hair follicles adjacent to the inflammation and necrosis, raising the possibility of infected hair follicles and pilonidal disease.”
Unlike the implications with the presumed Behçet’s, the patient’s localized disease process has no long-term health consequences. She is discharged with a diagnosis of labial ulcers with cellulitis, resolved urinary retention, and a pathological diagnosis of pilonidal disease.
Q: What considerations exist in the diagnosis of pilonidal disease?
A: ____________________________________________________________ ______________________________________________________________ ______________________________________________________________ ______________________________________________________________ ______________________________________________________________
Pilonidal disease
Pilonidal disease, this patient’s final diagnosis, is more common than Behçet’s disease. Men are affected more often than women, and ages range from puberty to the third decade of life with decreased frequency after the age of 45.
Pilonidal disease becomes a problem when “nests of hair” become infected. It is mostly found in the natal cleft but can present in almost any hairy area, mostly in the midline. Other examples include the umbilicus and scalp. There are also reports of acquired pilonidal disease from trauma in the hands of barbers, hair dressers, sheep shearers, and animal groomers. It is important to distinguish pilonidal disease from other disorders in the same region, such as anal fistulas, perirectal abscesses, and hidradenitis suppurativa.
Recurrences of pilonidal disease are quite common, with painful inflamed masses, abscesses, or sinus tracts. An acute abscess must be incised and drained. Hair removal by laser may prevent the growth of hair temporarily. For recurring infections, the definitive treatment is surgical. Procedures range from simple incision and curettage to excision of the area with plastic flap reconstruction. Antibiotics are not routinely needed, but they may be appropriate when cultures indicate specific microbes, when significant cellulitis is present, or for perioperative prophylaxis.
Q: What are the comparative likelihoods of pilonidal disease and hidradenitis suppurativa?
A: ____________________________________________________________ ______________________________________________________________ ______________________________________________________________
Could it be hidradenitis suppurativa?
Hidradenitis suppurativa is a possibility, but the clinical and pathological findings make pilonidal disease more likely. Hidradenitis suppurativa similar to pilonidal disease, but it usually is located in the axillary, perineal, or inguinal areas. Incidence is as high as 1 in 300, and it frequently begins with puberty.
Hidradenitis suppurativa differs from pilonidal disease in that the hair follicle becomes occluded due to a defect in the epithelium of the follicle’s terminal ends. This defect leads to trapping of bacteria and formation of firm, peasized nodules or cysts. Some associations between hidradenitis suppurativa and sex hormones have been proposed, but hormonal states of pregnancy, menstruation, and menopause have not shown clear association with hidradenitis suppurativa. Initial symptoms of hidradenitis suppurativa may be pruritus, erythema, and hyperhidrosis. Recurrences are common. Treatment includes medications such as antibiotics, antiandrogens, and retinoids, as well as surgical excision.