Teen girl presents with recurring sores
What’s your diagnosis?
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You are asked to see a 14-year-old girl for recurrent, draining sores in the bilateral groin areas for over a year. She did not want to tell her parents, fearing what they might think, even though she was not sexually active.
The lesions occur in cycles that begin with an erythematous papule and progress to an abscess and spontaneous drainage, then may continue to drain, either forming a sinus tract or heal over (Figure 1). The sores do not occur anywhere else on her body. When she finally told her mother, she was taken to her primary care physician, who cultured a draining lesion, growing methicillin-sensitive Staphylococcus aureus. She was treated with cefalexin and saw improvement, but after treatment, the lesions began to recur.
Her immunizations are up to date, and she has no other health problems. Her family history is positive for the mother having recurrent sores in the inframammary areas of both breasts as an adolescent, which lasted about a year. Otherwise, her family history is unremarkable, and she has had no foreign travel.
Her exam confirms the lesions noted earlier in an otherwise healthy-appearing adolescent girl.
What’s your diagnosis?
A. Granuloma inguinale
B. Hidradenitis suppurativa
C. Job syndrome
D. Recurrent folliculitis
Answer and discussion:
The answer is B, hidradenitis suppurativa (HS), a subacute chronic inflammatory condition often found in other family members, affecting the apocrine glands in the inguinal, inframammary and axillary areas of adolescents and young adults. The condition affects females more than males. Figure 2 shows an axillary case seen in a male. It may occur anywhere, favoring skin-to-skin contact with movement. This patient was featured in this column exactly 22 years ago (March 2001).
Since then, there has been growing evidence of a genetically driven autoinflammatory component to the disorder. The process begins with ductal obstruction, trapping bacteria (usually S. aureus) in the subcutaneous nutrient-rich environment, promoting the establishment of an infection. As the infection spreads, subcutaneous abscess formation occurs with spontaneous drainage and/or connects with adjacent abscesses via sinus tract formation. The disorder is often classified by five phenotypes — from simple lesions without deep nodules to those with furuncles, to scarring folliculitis, to cyst formation with acne lesions on the back and face, to those with other autoinflammatory syndromes.
Treatment includes systemic, topical antibiotics based on culture results and to address any underlying comorbidities. Often, surgical ablation is required. As such, most cases are best managed in a dermatology clinic.
Granuloma inguinale is a rare sexually transmitted infection in this country, caused by Klebsiella granulomatis. According to the CDC, it is most often seen in travelers to areas where it is more common, such as India, South Africa, South America and others. It produces nodular, painless ulcers in the inguinal/genital areas (Figure 3). Culture is difficult, leaving physicians to base their diagnosis on historic and clinical grounds. If laboratory diagnosis is necessary, dark staining a tissue specimen showing “Donovan bodies” (clumps of organisms within the phagocyte) may be done. Molecular techniques may also be available. Treatment of choice is with azithromycin, but doxycycline, ciprofloxacin or trimethoprim/sulfamethoxazole can also be used.
Job syndrome, also known as hyper-IgE immunodeficiency syndrome, results in severe dermatitis and recurrent skin infections (cellulitis) with abscesses, which are not restricted to the crural areas described in the case vignette. Sinopulmonary infections, bacteremia, sepsis and osteomyelitis are common complications in those with poor care. Figure 4 shows the skin of a patient with Job syndrome, with multiple scars from frequent infections.
Some facial features also are seen, such as facial asymmetry, broad nasal bridge and prominent forehead (Figure 5). The disorder is caused by an autosomal dominant signal transducer and activator of transcription 3 (STAT3) gene mutation. A recessive form with similar manifestations results from a mutation in the dedicator of cytokinesis 8 (DOCK8) gene. The mainstay of therapy is meticulous attention to skin care and early treatment of infections with antistaphylococcal antimicrobial agents. Immunology and infectious diseases specialists should be part of the management team.
Recurrent folliculitis is when hair follicles become inflamed due to minor, superficial infection. It is much less severe than HS and usually caused by microtrauma to the superficial layers of the skin, such as that caused by the rubbing of tight-fitting clothes or shaving. The treatment is basic skin hygiene, and patients should avoid tight-fitting clothes or shaving (Figure 6) areas that are prone to infection.
Columnist comments
Seventeen years ago, I was invited to give grand rounds and a keynote presentation on febrile exanthema at the annual Katz-Wilfert Update in Pediatric Infectious Diseases at Duke University School of Medicine. Dr. Catherine Wilfert (1936-2020) spent her career as a pediatric infectious diseases specialist at Duke, and her work treating HIV-infected pregnant women with zidovudine (the ACTG protocol 076) no doubt saved countless children from HIV infection. I never met Dr. Wilfert as she was out of the country during the meeting that year, but her husband Dr. Sam Katz was there, and I must say, at first, I was very nervous to be in the same room with this giant among ID specialists. As I took the lectern for my presentation, I saw Dr. Katz sitting front row center and briefly thought, “What have I done? Here I am about to talk about measles, with Sam Katz, the person with whom John Enders developed the measles vaccine, sitting right in front of me.” But I soon realized that he could not have been more gracious, and it made the rest of the trip a most enriching experience.
There have been many worthy testimonials written about Sam Katz since his death at 95 years of age on Oct. 31, 2022 — including one published in The New York Times and another from Duke University .
On behalf of my children and grandchildren, and all others who escaped measles, and the risk of potential death and severe complications of the disease, I would like to add my sincere thanks to Dr. Katz for his work on the measles vaccine, as well as his long history of educating the hundreds of fortunate students, residents and fellows, and the countless readers of his literature.
References:
CDC. Granuloma inguinale/donovanosis. https://www.cdc.gov/immigrantrefugeehealth/guidelines/domestic/sexually-transmitted-diseases/granuloma-inguinale-donovanosis.html. Accessed March 6, 2023.
Cooper ER, et al. J Infect Dis. 1996;doi:10.1093/infdis/174.6.1207.
A humble giant with broad shoulders. https://giving.dukehealth.org/why-give/meet-physicians-scientists/samuel-katz. Accessed March 6, 2023.
Sandomir R. Samuel L. Katz, a developer of the measles vaccine, dies at 95. The New York Times. https://www.nytimes.com/2022/11/05/health/samuel-katz-dead.html. Published Nov. 5, 2022. Accessed March 6, 2023.
For more information:
Brien is a member of the Healio Pediatrics Peer Perspective Board and an adjunct professor of pediatric infectious diseases at McLane Children's Hospital, Baylor Scott & White Health, in Temple, Texas. He can be reached at jhbrien@aol.com.
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