Pavone Piero, Praticò Andrea D, Falsaperla Raffaele, Ruggieri Martino, Zollino Marcella, Corsello Giovanni, Neri Giovanni
Unit of Pediatrics and Pediatric Emergency, University Hospital "Policlinico-Vittorio Emanuele", Catania, Italy.
Section of Pediatrics and Child Neuropsychiatry. Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy.
Ital J Pediatr. 2015 Aug 5;41:55. doi: 10.1186/s13052-015-0161-3.
Hypertrichosis is defined as an excessive growth in body hair beyond the normal variation compared with individuals of the same age, race and sex and affecting areas not predominantly androgen-dependent. The term hirsutism is usually referred to patients, mainly women, who show excessive hair growth with male pattern distribution.Hypertrichosis is classified according to age of onset (congenital or acquired), extent of distribution (generalized or circumscribed), site involved, and to whether the disorder is isolated or associated with other anomalies. Congenital hypertrichosis is rare and may be an isolated condition of the skin or a component feature of other disorders. Acquired hypertrichosis is more frequent and is secondary to a variety of causes including drug side effects, metabolic and endocrine disorders, cutaneous auto-inflammatory or infectious diseases, malnutrition and anorexia nervosa, and ovarian and adrenal neoplasms. In most cases, hypertrichosis is not an isolated symptom but is associated with other clinical signs including intellective delay, epilepsy or complex body malformations.A review of congenital generalized hypertrichosis is reported with particular attention given to the disorders where excessive diffuse body hair is a sign indicating the presence of complex malformation syndromes. The clinical course of a patient, previously described, with a 20-year follow-up is reported.
多毛症的定义是，与同年龄、种族和性别的个体相比，身体毛发过度生长，超出正常范围，且累及的部位并非主要受雄激素影响。多毛症一词通常用于指主要为女性的患者，这些患者表现出具有男性分布模式的毛发过度生长。多毛症根据发病年龄（先天性或后天性）、分布范围（全身性或局限性）、受累部位，以及该病症是孤立存在还是与其他异常相关进行分类。先天性多毛症较为罕见，可能是一种孤立的皮肤病症，或是其他病症的一个组成特征。后天性多毛症更为常见，继发于多种原因，包括药物副作用、代谢和内分泌紊乱、皮肤自身炎症或感染性疾病、营养不良和神经性厌食症，以及卵巢和肾上腺肿瘤。在大多数情况下，多毛症并非孤立症状，而是与其他临床体征相关，包括智力发育迟缓、癫痫或复杂的身体畸形。本文报道了对先天性全身性多毛症的综述，特别关注那些以全身弥漫性过多毛发为标志，提示存在复杂畸形综合征的病症。还报道了一名先前描述过的患者长达20年的随访临床过程。
