INTRODUCTION
Hidradenitis suppurativa (HS) is a chronic inflammatory disease of the follicular unit in intertriginous areas, including the axillae, inguinal and anogenital areas.1 Clinical manifestations include the presence of double-headed comedones, painful nodules that rupture and drain, purulent discharge, sinus tract formation and scarring. HS occurs in approximately 2-4% of the population, predominantly in women (2:1), and often presents in the third decade of life.1-3
Reports of HS in the pediatric population are limited.1,2,4 Approximately 7% of patients have onset of HS before age 13, and 36% of cases present between age 13 and 20 years.1-4 Prepubertal onset is estimated to occur in less than 2% of patients and associated with a more severe course, widespread distribution and higher risk of endocrine abnormalities.3,5 Co-morbidities in pediatric patients include adrenal hyperplasia, premature adrenarche, obesity, precocious puberty and metabolic syndrome.1-3 A positive family history of HS is more commonly reported in childhood-onset disease with an earlier onset of presentation.1,5 The etiology of HS is not well understood. The disease tends to favor apocrine-bearing skin, but histopathological evaluation suggests disease centers around the pilosebaceous unit, with a follicular structure abnormality leading to dilation and rupture of the follicular tract and subsequent inflammatory cascade.6 A genetic predisposition has been considered; impaired notch signaling in hair follicles due to heterozygous mutations in NCSTN, PSEN1 or PSENEN may be significant in the development of the nodules. However, an exact mechanism and relationship with early-onset disease has not been clarified.1,7 A hormonal influence is suggested by the known female preponderance, flares with puberty and menstruation, and association with endocrine abnormalities such as polycystic ovarian syndrome (PCOS) and obesity in some adolescents. HS is most commonly classified according to Hurley stages based on the presence and confluence of nodules, sinus tracts and scars.1,2,8
Treatment of HS is guided by severity and disease progression. There are no established guidelines for pediatric HS; however,
Reports of HS in the pediatric population are limited.1,2,4 Approximately 7% of patients have onset of HS before age 13, and 36% of cases present between age 13 and 20 years.1-4 Prepubertal onset is estimated to occur in less than 2% of patients and associated with a more severe course, widespread distribution and higher risk of endocrine abnormalities.3,5 Co-morbidities in pediatric patients include adrenal hyperplasia, premature adrenarche, obesity, precocious puberty and metabolic syndrome.1-3 A positive family history of HS is more commonly reported in childhood-onset disease with an earlier onset of presentation.1,5 The etiology of HS is not well understood. The disease tends to favor apocrine-bearing skin, but histopathological evaluation suggests disease centers around the pilosebaceous unit, with a follicular structure abnormality leading to dilation and rupture of the follicular tract and subsequent inflammatory cascade.6 A genetic predisposition has been considered; impaired notch signaling in hair follicles due to heterozygous mutations in NCSTN, PSEN1 or PSENEN may be significant in the development of the nodules. However, an exact mechanism and relationship with early-onset disease has not been clarified.1,7 A hormonal influence is suggested by the known female preponderance, flares with puberty and menstruation, and association with endocrine abnormalities such as polycystic ovarian syndrome (PCOS) and obesity in some adolescents. HS is most commonly classified according to Hurley stages based on the presence and confluence of nodules, sinus tracts and scars.1,2,8
Treatment of HS is guided by severity and disease progression. There are no established guidelines for pediatric HS; however,
