Sechi Andrea, Guglielmo Alba, Patrizi Annalisa, Savoia Francesco, Cocchi Guido, Leuzzi Miriam, Chessa Marco A
Division of Dermatology, Sant'Orsola-Malpighi Polyclinic, Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Italy.
Neonatology Unit, Sant'Orsola-Malpighi Polyclinic, Department of Medical and Surgical Sciences, University of Bologna, Italy.
Dermatol Pract Concept. 2019 Jul 31;9(3):187-194. doi: 10.5826/dpc.0903a03. eCollection 2019 Jul.
Hidradenitis suppurativa (HS) is a chronic, inflammatory, recurrent skin disease of the pilosebaceous unit characterized by protean manifestations. Several studies have found an increased incidence and earlier presentation of this disease in patients carrying trisomy 21. Patients with Down syndrome (DS) have a higher risk of developing a wide range of cutaneous manifestations, including HS and chronic folliculitis. Recently, disseminate recurrent folliculitis (DRF) has been reported as an atypical monosymptomatic feature of HS at its onset.
To assess the prevalence of HS and DRF by comparing a cohort of patients carrying trisomy 21 vs pediatric controls.
A retrospective 2-year monocentric clinical study was performed by collecting clinical data of 131 patients with DS, aged 4-36 years, followed at the Dermatology Unit and Down Syndrome Regional Center of Bologna University. Data were matched with those coming from 12,351 pediatric controls.
In DS patients, DRF and HS showed a prevalence of, respectively, 6.8% and 24.4%, while 5.3% of patients presented both diseases. In the control group the prevalence for HS+ and DRF+ was 0.5% and 1.2%, respectively, with a 0.14% of overlap cases. The association between HS and DRF proved to be statistically significant in both groups (P < 0.05). In the DS cohort the mean age of symptoms onset was 15.67 (SD: 2.29) years for HS and 13.11 (SD: 4.93) years for DRF. Buttocks were the most frequently affected body area for DRF followed by the inguinocrural area, while in HS buttocks were less frequently involved than groins and upper thighs.
Because of the later onset of HS, patients with DRF at an early age should be monitored for the possible onset of HS in the apocrine-bearing areas.
化脓性汗腺炎（HS）是一种慢性、炎症性、复发性毛囊皮脂腺单位皮肤病，表现形式多样。多项研究发现，21三体综合征患者中该病的发病率增加且发病年龄提前。唐氏综合征（DS）患者发生包括HS和慢性毛囊炎在内的多种皮肤表现的风险更高。最近，播散性复发性毛囊炎（DRF）被报道为HS起病时的一种非典型单症状特征。
通过比较21三体综合征患者队列与儿科对照，评估HS和DRF的患病率。
进行了一项为期2年的回顾性单中心临床研究，收集了博洛尼亚大学皮肤科和唐氏综合征区域中心随访的131例4至36岁DS患者的临床数据。数据与来自12351名儿科对照的数据进行匹配。
在DS患者中，DRF和HS的患病率分别为6.8%和24.4%，而5.3%的患者同时患有这两种疾病。在对照组中，HS+和DRF+的患病率分别为0.5%和1.2%，重叠病例为0.14%。HS和DRF之间的关联在两组中均具有统计学意义（P<0.05）。在DS队列中，HS症状的平均发病年龄为15.67（标准差：2.29）岁，DRF为13.11（标准差：4.93）岁。DRF最常累及的身体部位是臀部，其次是腹股沟区，而HS累及臀部的频率低于腹股沟和大腿上部。
由于HS发病较晚，对于早期患有DRF的患者，应监测其大汗腺分布区域是否可能发生HS。
