15-year-old male presents with recurrent abscesses in both axillae
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A 15-year-old male presents to your office with recurrent abscesses in both axillae for the last 8 months. The lesions begin as red bumps that go on to form abscesses that ultimately drain. The patient has been to other providers, mostly in EDs or urgent care clinics, who have performed incision and drainage on several prior occasions. Occasionally, oral antimicrobial therapy has been used without drainage, but the lesions soon reappear. The patient is somewhat obese but otherwise fairly healthy and is not sexually active. Interestingly, the patient’s sister and mother have had similar problems occurring in the groin over the years.
Examination reveals normal vital signs and obesity, with the only significant positive finding being related to the chief complaint, which reveals each axillary area to have some old scars and several new and actively draining sores, mostly on the left side as shown in Figures 1 and 2. There are no lab tests or imaging results.
What’s your diagnosis?
A. Recurrent folliculitis
B. Hidradenitis suppurativa
C. Job syndrome
D. Hyper-IgE syndrome
Case Discussion
This is a case of hidradenitis suppurative (B), which is a disorder of apocrine glands observed only after puberty, implying that hormonal changes may play a role. Additionally, my old friend, Meg Fisher, MD, chair of the department of pediatrics at Monmouth Medical Center in Long Branch, New Jersey, recently drew my attention to developments in recent years that implicate the immune response may be in part responsible.
The problem is thought to begin with ductal obstruction with keratin, thereby trapping bacteria (usually Staphylococcus aureus) in a nutrient-rich environment, leading to infection. The infection progresses, with subcutaneous spread and abscess formation resulting in spontaneous drainage and oftentimes sinus tract formation with scar formation as well. There is often a family history of the same condition, which occurs most frequently in adolescent females and usually in the inguinal or inframammary areas, whereas in males, it tends to occur in the axillary or perianal areas. It is more common in those with acne, and in adults, it is associated with smoking.
Treatment is usually started with antimicrobial therapy and often with surgical drainage. Some lesions will “burn out” over time, leaving a scar, but oftentimes, surgical ablation of the glands involved is necessary by marsupialization. I always recommend referring to the patient a dermatologist for the best therapy, which may include methotrexate or other immune modulators. A different patient was featured in the March 2001 column (Figure 3) for those who want to read more about this.
Recurrent folliculitis is the formation of microabscesses at the hair follicle, also usually due to S. aureus, but may be mixed with other organisms. Caused in part by microtrauma of the skin, usually from shaving, tight-fitting clothing (Figure 4) with repetitive activity of rubbing the skin and prolonged wetness, recurrent folliculitis is usually easily treated by discontinuing or removing the cause of this microtrauma.
Job syndrome and hyper-IgE immunodeficiency syndrome are essentially the same thing — an immune deficiency syndrome that was originally named and described in the mid-1960s by Davis and colleagues. About a decade later, Rebecca Buckley, MD, made the association with extraordinarily high IgE levels, and renamed the condition to what it is known today as hyper-IgE immunodeficiency syndrome.
This syndrome is usually associated with chronic dermatitis, with recurrent episodes of S. aureus infections with abscess formation, septicemia, deeper infections with bone and joint involvement and sinopulmonary infections. In addition, there are also characteristic facial features, such as frontal bossing, asymmetric face, prominent chin and broad nasal bridge and alae. Although most of these cases are sporadic in nature, some appear to have an autosomal dominant or autosomal recessive pattern and are frequently associated with a STAT3 gene mutation. Treatment requires good skin care and vigilance to the increased risk of serious infections and prompt therapy.
Disclosure: Brien reports no relevant financial disclosures.
Columnist Comment: We are off to what may be shaping up to be a difficult influenza season. If you see patients, you will probably be exposed. Be sure to get your influenza immunization as soon as possible, if not done already.