Schoch Jennifer J, Tolkachjov Stanislav N, Cappel Jonathan A, Gibson Lawrence E, Davis Dawn Marie R
Department of Dermatology, Mayo Clinic, Rochester, Minnesota.
Division of Anatomic Pathology, Mayo Clinic, Rochester, Minnesota.
Pediatr Dermatol. 2017 Jan;34(1):39-45. doi: 10.1111/pde.12990. Epub 2016 Oct 4.
BACKGROUND/OBJECTIVES: Pyoderma gangrenosum (PG) is a neutrophilic dermatosis rarely seen in children. Its features have not been well characterized in children. We sought to characterize the clinical features, etiologic associations, and treatment of PG in children younger than 18 years.
We performed a retrospective review of children younger than 18 years with PG at the Mayo Clinic from January 1976 to August 2013.
Thirteen children with PG were identified (n = 8; 62% female). All had ulcerations, with 62% having pustular lesions. Sites of involvement included the trunk (77%), lower extremities (77%), upper extremities (38%), and head and neck (38%). Nine (69%) had an underlying comorbidity, including seven with Crohn's disease (54%), one with juvenile idiopathic arthritis (8%), and one with pyogenic arthritis, pyoderma gangrenosum, and acne syndrome (8%). Treatments included topical or local care (92%) and systemic therapies (85%) such as oral corticosteroids (62%) and sulfasalazine or related 5-aminosalicylate drugs (46%). The clinical course did not correlate with that of the underlying systemic disease and response to treatment varied.
Pediatric PG has a more varied anatomic distribution and a greater predominance of pustular lesions than PG in adults and a strong association with inflammatory bowel disease.
背景/目的:坏疽性脓皮病(PG)是一种在儿童中罕见的嗜中性皮病。其特征在儿童中尚未得到充分描述。我们旨在描述18岁以下儿童PG的临床特征、病因关联及治疗情况。
我们对1976年1月至2013年8月在梅奥诊所诊断为PG的18岁以下儿童进行了回顾性研究。
共确定了13例PG患儿(8例女性,占62%)。所有患儿均有溃疡,62%有脓疱性损害。受累部位包括躯干(77%)、下肢(77%)、上肢(38%)和头颈部(38%)。9例(69%)有潜在合并症,其中7例患有克罗恩病(54%),1例患有幼年特发性关节炎(8%),1例患有化脓性关节炎、坏疽性脓皮病和痤疮综合征(8%)。治疗方法包括局部或局部护理(92%)和全身治疗(85%),如口服糖皮质激素(62%)和柳氮磺胺吡啶或相关的5-氨基水杨酸药物(46%)。临床病程与潜在全身性疾病的病程无关,对治疗的反应各不相同。
儿童PG的解剖分布比成人PG更多样化,脓疱性损害更常见,且与炎症性肠病密切相关。