Cas report
We report the case of an 8-year-old Turkish girl who consulted for painful and deep inflammatory nodular skin lesions on her inguinal folds, evolving for several months. She had no specific history except moderate obesity. There was a family history of type II diabetes in the grandmother. The patient has no clinical and biological signs of early puberty. The diagnosis of Hurley I Hidradenitis suppurativa was made. A topical clindamycin treatment to be applied twice a day was iniciated, allowing a month later, a significant improvement of the lesions.
Introduction
Hidradenitis suppurativa (HS) is a chronic, recurrent, inflammatory disease with a high physical and psychological burden. It is a disorder of the infundibular segment of the pilosebaceous unit, characterized by subcutaneous nodules, abscesses, sinus tracts and scar formation on the intertriginous and apocrine-bearing areas, such as the axillae, inguinal and perianal areas.
Epidemiology
HS is quite rare in young and prepubertal children, with onset generally occurring well after puberty, typically between 20 and 24 years of age (1). Prepubertal onset, before 11 years of age, is estimated to occur in 2% of patient with HS, while 36% debut between the ages of 11 and 20 years (2). HS is more common in women than in men by a ratio of 2:1. In the pediatric population, it is also predominant in girls, but the prevalence is unknown. The youngest child reported with HS was 5 years old (3).
Pathogenesis
Children with HS are more prone to having underlying hormonal disorders than adult patients. The diagnosis of HS in children must search for a precocious puberty. Some cases of adrenal hyperplasia, premature adrenarche, obesity and metabolic syndrome have been reported. An endocrinologic evaluation is recommended.
Genetic susceptibility may account for early onset of the disease. A positive family history of HS is also more common in early-onset HS than in onset in adulthood (56% vs 35%) (2).
Clinical features
Tender subcutaneous nodules and double-headed open comedones involving the intertriginous areas are characterizing manifestations of pediatric HS (3). As the disease progresses, deep dermal abscesses drain a foul-smelling purulent discharge, leading to the sinus tracts, and fibrotic scars, retracing the evolution of HS in adulthood. Early onset is usually associated with more widespread disease (2).
Treatment
Treatment should be tailored on disease severity and progression. Education of patients and their families regarding the diagnosis, the chronic nature of HS, and therapeutic modalities is key to optimal disease management and adherence to medical recommendations. It is essential for patients to understand that there is no cure for HS and that the goals of therapy in children are to alleviate pain, minimize inflammation and scarring, prevent disease progression, and postpone the need for extensive surgery.
The management principles include the following:
- correction whenever possible of the metabolic/endocrine disturbance
- the promotion of overall health, including weight control
- symptom management (eg.pain)
- medical treatment (3,4)
For localized, mild HS in children (Hurley I), conservative therapy is advised. The most frequently used topical treatment is clindamycin 1% , applied twice daily for 3 months. Chlorehexidine or zinc pyrithione washes are used as adjuvants. Azelaic acid 15% or 20% twice daily for 2 months is effective and safe for pediatric use. A beneficial effect is described for papules and pustules, with a limited effect on deeper lesions, such as nodules and abscesses.
Oral antibiotics can be used in children with moderate HS (Hurley II) or as second-line therapy in patients with mild HS refractory to topical treatment. The most common antibiotic prescribed in the pediatric population is oral clindamycin (10-25mg/kg/day) administered every 6-8 hours. The combination of clindamycin 300mg and rifampicin 300mg has proven effective in a small series with no significant side-effects. Oral tetracyclines are a class of broad-spectrum antibiotics and represent a key treatment for adults HS. Their use in pediatric arena is considered safe in children ≥ 10 years of age, in the setting of HS refractory to a topical approach. Doxycycline will be used in children younger.
Currently, adalimumab at a dose regimen of 80 mg at week 0, followed by 40 mg every other week, is indicated for the treatment of active moderate to severe HS from 12 years of age with an inadequate response to conventional systemic therapy.
Other potential treatment options in children include botulinum toxin A and photodynamic therapy. Laser therapy with fractionated CO2 laser could be used to address residual scarring. Nd:YAG lasers as well as intense pulsed light therapy for hair removal might have a basis in preventing HS.
If the aforementioned treatments prove insufficient or if the disease progesses to Hurley III, surgical treatment (deroofing and excisions) may be considered.
In a recent case series the use of short-term oral azitrhomycin for control HS flares, in association with routine use of oral zinc and/or topical clindamycin was considered as a useful and save approach in the pediatric population (4).
Conclusion
HS is distressing condition for many patients. Early recognition and treatment therefor is vital to minimize the long-term effects of this disease throughout in the life course.
References
- Scheinfeld N. Hidradenitis suppurativa in prepubescent and pubescent children. Clin dermatol. 2015;33:316-319
- ckers IE, vander Zee HH, Boer J, Prens EP. Correlation of early-onset hidradenitis suppurativa with stronger genetic susceptibility and more widespread involvement. J Am Acad Dermatol 2015;72:485-488
- Liy-WongC, Pope E, Lara-Corrales I. Hidradenitis suppurativa in the pediatric population. J Am Acad Dermatol 2015; 73:36-41
- Offidani A, Molinelli E, Sechi A, Brisigotti V, Campanati A, Raone B, Neri I, Patrizi A. JEADV 2019; 33:28-31
Ch. Roquet, A Bulinckx
Service de dermatologie
Grand Hôpital de Charleroi