March 2001

A 14-year-old female is referred to the infectious disease clinic for evaluation of recurrent pustules and abscesses on her upper thighs. This problem was first noticed about five years earlier.

These lesions would typically begin as simple maculopapular lesions that would progress to pustules and grow into larger abscesses with spontaneous drainage.

She has undergone multiple courses of oral and topical antibiotics. The lesions would resolve on oral antibiotics, but reoccur soon after finishing the course.

She did not tell her parents about this problem for several years because of embarrassment and would wear pads or other material to keep from staining her underwear with pus.

Her past medical history is unremarkable, and no one else in the family has any similar problems. Her only activity outside of school is bird hunting with her father, which she does frequently. She has traveled across the border to Mexico on several hunting trips, but that does not seem to be associated with her skin problem.

Her immunizations are up to date. Her family history is unremarkable and she denies being sexually active.

Examination reveals numerous lesions in various stages from maculopapular to open abscesses on the inner aspect of the upper thighs and inguinal area (figures 1 & 2).

The rest of her exam was normal. There are no other skin problems, including acne. The drainage from one lesion was sent for culture. No other tests were done.

What's Your Diagnosis?

A.) Recurrent folliculitis

B.) Hidradenitis suppurativa

C.) Job's syndrome

D.) Lymphogranuloma venereum

Answer

The condition described in this case is hidradenitis suppurativa. This is an unusual condition of the apocrine glands, which become recurrently infected, developing draining abscesses, and ultimately, much fibrosis.

These lesions occur in post-pubertal patients of both sexes, often in those with acne, and tends to be located in the crural areas, such as the perianal area in men, and the axillary, inguinal and inframammary areas in women. Experimental data from Shelly & Cahn (Arch Dermatol 1955;72:562), suggests that this process begins with obstruction of the apocrine duct with keratin, with resultant dilation of the duct and trapping of bacteria in the process, setting the stage for infection. The infection may break through and spread subcutaneously to other adjacent glands, producing a large abscess (figure 3) that eventually drains out, forming a sinus tract as this process recurs.

The cases that I have seen have all been in adolescent females, mostly with inguinal disease, followed by axillary involvement as seen in figures 4 through 7. In each case, these girls hid the fact that something was wrong for long periods (often for years); embarrassed to bring it even to their parents' attention. They would wear absorbent padding over these areas to conceal the drainage and odor. When treatment was finally obtained, and their condition improved, it was as though a great weight was lifted from their shoulders. However, treatment at this stage is difficult at best. The natural history of these lesions is to sometimes "burn out" with resultant scar formation (figure 8), but they usually continue to recur. The acute infectious process is usually responsive to a variety of systemic antibiotics; however, I feel the drainage should be cultured and treated based on the organism recovered.

In the case of this patient, her culture grew a large amount of Staphylococcus aureus. In general, these patients should be co-managed with a dermatologist, and possibly a plastic surgeon, as these chronic lesions can best be cured with excision. To read more about this unusual and complicated condition, I recommend Harry J. Hurley's chapter on Diseases of The Apocrine Sweat Glands in Moschella and Hurley's textbook, Dermatology 1992 edition, pages 1506-1513.

Simple, recurrent folliculitis is a much less serious, superficial local infection of the hair follicle, producing small, pustular lesions with an erythematous base. The degree of infection can range from minimal to fairly significant (figures 9 and 10). Like hidradenitis suppurativa, folliculitis is often aggravated by skin trauma, usually friction or shaving, or compression of tight-fitting clothing, giving colonizing bacteria an enhanced opportunity to invade. The treatment is to fix the underlying cause, such as microtrauma to the skin and try to reduce the surface colonization of bacteria. Topical, and occasionally systemic antibiotics, are needed to treat the condition. Often this can be complicated by the patient being colonized with a more virulent strain of Staphylococcus, which can further aggravate the condition and contribute to the recurrent nature of it.

Job's syndrome is a very uncommon immunodeficiency condition involving defective leukocyte migration, but of uncertain etiology, that was first described by Davis & Schaller (Lancet. 1966;1:1013-1015 in 1966. The name was derived from the biblical Book of Job, which describes Job being afflicted with boils from his feet to his head by Satan. It is also associated with very high levels of IgE and marked eosinophilia. It appears to be the same condition as the "hyperimmunoglobulinemia E syndrome" first reported in Pediatrics (49:59) in 1972 by Rebecca Buckley. he clinical features include recurrent infections of the skin, producing deep subcutaneous abscesses. It is also associated with recurrent sinopulmonary infections. These infections are predominately due to S. aureus but other bacteria, Candida,and herpes have also been seen. A recent study by Borges, Augustine and Hill Defective IL-12/IFN-gamma pathway in patients with hyper-IgE syndrome. J Ped. 2000;136:176) indicates that cytokine dysregulation appears to play a role.

Patient histories with Job's syndrome will usually date back to early infancy. As one can imagine, treatment involves aggressive antimicrobial therapy and possibly intravenous immune globulin. The differences in this and the patient presented are the age of onset, the restricted location of the lesions to the inguinal area, and the lack of any history of sinopulmonary disease.

Lymphogranuloma venereum is a venereal disease caused by Chlamydia trachomatis and results in ulcerative lesions of the genitalia with associated inguinal lymphadenitis (figure 11). It is most common in blacks and males, and has a worldwide distribution. Diagnosis is by culture and/or by one of several antigen detection techniques (DFA, EIA, PCR, or DNA probe). Treatment is best with doxycycline, or in children, erythromycin. The patient presented had neither genital lesions nor adenopathy. However, Granuloma inguinale is another sexually transmitted disease that does produce granulomatous ulcers, usually of the genitalia (figures 12 and 13). It is caused by a gram-negative bacillus called Calymmatobacterium granulomatis however this is very rare in the United States. These painless ulcers should also be easy to distinguish from the pustular lesions of hidradenitis suppurativa. I am normally a very trusting person, but when it comes to the history of sexual activity in adolescents, I leave it in the differential just in case.

Acknowledgement: Thanks to Susan Nickel, MD, Department of Pediatrics, Scott & White Children's Health Center, Temple, Texas, for her help with this case.

For Your Information:

• James H. Brien, DO, Pediatric Infectious Disease, Scott and White's Children's Health Center and Texas A&M University, College of Medicine, Temple, Texas. E-mail: jhbriend@aol.com