Cartron A M, Buccine D, Treichel A M, Lee C R, Moss J, Darling T N
Pulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA.
Department of Dermatology, Uniformed Services University of the Health Sciences, Bethesda, MD, USA.
J Eur Acad Dermatol Venereol. 2021 May;35(5):1226-1229. doi: 10.1111/jdv.17161. Epub 2021 Feb 23.
Tuberous sclerosis complex (TSC) is a hamartoma syndrome characterized by multiple skin lesions, such as angiofibromas, shagreen patch and miliary fibromas (MiF).
To determine the clinical and histological features of MiF.
A retrospective analysis was conducted on 133 adults with TSC. Photography was used to characterize the appearance and location of MiF. Histological features in five skin samples from four individuals were evaluated by a board-certified dermatopathologist.
MiF were observed in 19 of 133 (14%) individuals with TSC. MiF were 1- to 3-mm skin-coloured, sessile papules scattered on the back and rarely buttocks or thighs. Most were scattered in a bilaterally symmetric distribution, but others were asymmetric or associated with a shagreen patch. Histological features of MiF included expansion of the papillary and periadnexal dermis with variable hamartomatous abnormalities involving adjacent epithelial components.
MiF are distinct from other cutaneous lesions in TSC such as shagreen patches and angiofibromas. Recognition of this entity is important in defining the spectrum of TSC disease and reassuring individuals with TSC that these lesions are benign.
结节性硬化症（TSC）是一种错构瘤综合征，其特征为出现多种皮肤损害，如血管纤维瘤、鲛鱼皮斑和粟丘疹（MiF）。
确定粟丘疹的临床和组织学特征。
对133例成年结节性硬化症患者进行回顾性分析。采用摄影来描述粟丘疹的外观和位置。由一名获得委员会认证的皮肤病理学家评估来自4例个体的5份皮肤样本的组织学特征。
在133例（14%）结节性硬化症患者中，有19例观察到粟丘疹。粟丘疹为1至3毫米大小的肤色、无蒂丘疹，散在于背部，很少见于臀部或大腿。大多数呈双侧对称分布，但其他的则不对称或与鲛鱼皮斑相关。粟丘疹的组织学特征包括乳头层和毛囊周围真皮层扩张，并伴有累及相邻上皮成分的不同错构瘤异常。
粟丘疹与结节性硬化症中的其他皮肤损害如鲛鱼皮斑和血管纤维瘤不同。认识到这一实体对于界定结节性硬化症疾病谱以及让结节性硬化症患者放心这些损害是良性的很重要。
