Hidradenitis suppurativa in ChildSee also in: Cellulitis DDx,Anogenital
Alerts and Notices
SynopsisHidradenitis suppurativa (HS), or acne inversa, is a chronic destructive inflammatory disorder of the terminal follicular epithelium. While the pathogenesis is unclear, it is thought that follicular rupture initiates interaction between the follicular microbiome and innate immune system, triggering an inflammatory response. HS is more common in women, adults between the third and fourth decades of life, Black individuals, and the socioeconomically disadvantaged. With a prevalence of up to 1% in some population-based studies, HS is a common disease.
The diagnosis is clinical and based on 3 criteria:
- Typical lesions: nodules, abscesses, and tunnels (previously referred to as sinuses and fistulae)
- Recurrence: more than 2 lesions over a 6-month period
- Typical locations: primarily intertriginous
Mechanical irritation such as by friction from tight clothing or shaving is often reported as a trigger. For many women, the week before menses can trigger disease flares, and pregnancy and the postpartum period can be associated with either disease improvement or flaring.
Obesity and cigarette smoking are associated with HS severity but are unlikely to be the main trigger of disease in most patients. Prevalence of metabolic syndrome, major cardiovascular events, cardiac death, and diabetes (type 1 or type 2) are increased in HS compared to the general populations. Some of the most frequently associated comorbidities are related to mental health, and depression, anxiety, and risk of suicide are major burdens for the population. Regional ileitis (Crohn disease) has a statistical association with HS, while ulcerative colitis does not.
HS is often accompanied by the other disorders of the "follicular occlusion tetrad," which includes acne conglobata, dissecting cellulitis, and pilonidal sinus. Less frequent associations to keep in mind are increased risk of polycystic ovarian syndrome and lymphoma. People with Down syndrome are known to be at increased risk of HS.
A positive family history is reported in 30%-50% of patients in recent cohorts, and familial risk studies indicate a 20-fold risk of disease development for those with an affected first-degree relative. Pathogenic variants affecting the gamma-secretase complex genes PSENEN, PSEN1, and NCSTN have been reported in familial cases.
Associated syndromes include PASH (pyoderma gangrenosum, acne, and hidradenitis suppurativa), PAPASH (pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa), and PsAPASH (psoriatic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa), and these may in some cases be associated with mutations in PSTPIP1.
Pediatric patient considerations: HS usually presents after puberty and is more common in girls. In children presenting with early-onset disease, there is a strong association with widespread involvement, hormonal imbalance, and a strong family history. As in adults, higher rates of coexisting obesity, anxiety disorders, acne vulgaris, and acne conglobata are reported. In patients presenting younger than 11 years, signs of precocious puberty may be observed.
L73.2 – Hidradenitis suppurativa
59393003 – Hidradenitis suppurativa
Differential Diagnosis & Pitfalls
- Epidermoid cysts
- Abscesses, including recurrent Staphylococcus aureus infection and CA-MRSA
- Pilonidal sinus (gluteal cleft)
- Ulcerative colitis (genital region)
- Pyoderma gangrenosum
- Bartholin gland abscess (genital region)
- Sebaceous adenitis
- Crohn disease (perianal)
- Perianal fistula (perianal)
- Granuloma inguinale (genital region)
- Lymphogranuloma venereum (genital region)
- Cat-scratch disease
Patient Information for Hidradenitis suppurativa in Child
OverviewHidradenitis suppurativa is a chronic condition of clogged sweat glands that leads to inflammation of the skin in areas such as the groin, underarms, underneath the breasts, and in between the buttocks. It most commonly appears as multiple large nodules (solid, raised bumps), abscesses (red, swollen, warm, tender bumps or lumps with pus inside), and tunnels (holes in the skin that may contain fluid such as pus) in these areas. The nodules and abscesses gradually get larger and drain pus. After multiple bouts of this cycle of plugging, enlargement, and drainage, there may be tunnel formation under the skin and scarring. Pain is the most common symptom, but individuals with hidradenitis suppurativa also report itchy lesions, a foul odor coming from lesions when they drain pus, feeling tired, and joint pain.
While there is no cure for hidradenitis suppurativa, you can work with your medical professional to treat existing lesions and prevent new ones.
Who’s At RiskHidradenitis suppurativa usually starts soon after puberty and continues into adult life. It is more common in women and in Black individuals. Hair removal from shaving or using depilatories, deodorants, and irritation from anything rubbing against the affected area can worsen the condition. Hidradenitis suppurativa is often associated with smoking, obesity, diabetes, and hormonal changes. Additionally, it often runs in families.
Signs & SymptomsThe groin and underarms are often affected. There may also be lesions between the buttocks or under the breasts due to friction from skin rubbing together, which is why people who are obese are more predisposed to this disease.
The lesions of hidradenitis suppurativa are firm, tender bumps. In lighter skin colors, the lesions are often pink or red. In darker skin colors, the lesions may appear brown, purple, or gray. There may also be tunneling under the skin and scar formation. There may be pairs of blackheads within scars, and the lesions may drain pus. Hidradenitis suppurativa lesions are rarely seen on the trunk of the body or on the scalp or legs.
Some people may experience only one or a few lesions, while others may have severe disease, affecting many areas.
People with hidradenitis suppurativa may experience depression and anxiety, especially if the disease is severe or chronic.
Self-Care GuidelinesMake sure to wash any inflamed, draining areas of hidradenitis suppurativa with antibacterial soap, and then apply an antibiotic ointment (Neosporin) and clean bandages. If there is a large amount of drainage, change the gauze pads and dressings often. Warm compresses and ibuprofen (Advil, Motrin) can help reduce the swelling. Avoid wearing tight-fitting clothing to help prevent further irritation.
Weight loss may decrease lesions by decreasing skin folds and, thus, friction on the skin. Smoking should be avoided.
When to Seek Medical CareSome mild cases of hidradenitis suppurativa can be treated with self-care measures. Healing of individual lesions takes about 1-2 weeks. Make sure to seek medical care if you are not able to control the pain with ibuprofen and warm compresses or if the condition is making you feel sad or anxious. Medical treatment may be necessary if the condition appears in multiple areas or continues to come back (recur). If there is excessive pain, excessive drainage, red streaking, you have a fever, or there are any other factors that may indicate there is an infection, see your medical professional. If you have been given antibiotics and the area does not improve within a few days, return to your medical provider.
TreatmentsYour medical professional may prescribe a topical antibiotic lotion or swab for mild areas, or they may prescribe a long course of oral antibiotics. You may be given an injection of steroids into the deep, painful hidradenitis suppurativa lesions. If hormonal fluctuations are thought to be contributing to the condition, you may be prescribed hormonal therapy. Other treatments include a retinoid such as acitretin (Soriatane) and biologic therapies such as adalimumab (Humira).
Surgical removal is often the ideal treatment for severe cases of hidradenitis suppurativa with scarring and tunneling.
Hidradenitis suppurativa in ChildSee also in: Cellulitis DDx,Anogenital