Department of Pathophysiology and Transplantation, Università degli Studi di Milano.
Dermatology Unit, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Int J Dermatol. 2021 Jan;60(1):33-38. doi: 10.1111/ijd.14930. Epub 2020 May 13.
Hyperkeratosis lenticularis perstans (HLP), or Flegel's disease (FD), is a rare disorder of keratinization first described in 1958 by Flegel. HLP is characterized by asymptomatic small papules distributed symmetrically on the dorsal feet and lower part of the legs. It typically presents in the fourth or fifth decade of life. FD has been reported more commonly in women, and little is known about the pathogenesis of this rare dermatosis. Histological examination is mandatory to confirm the diagnosis, and the characteristic features are: focal compact hyperkeratosis, thinned stratum malpighii, epidermal atrophy, and a dense, band-like lymphocytic infiltrate in the papillary dermis. The treatment of FD is challenging, and a fully effective standard therapy does not exist. We report four cases of HLP that have been diagnosed at the Dermatology Unit of IRCCS Ca' Granda Ospedale Maggiore Policlinico in Milan, Italy, and a literature review is enclosed.
持久性层状角化过度症(HLP),或弗莱格尔病(FD),是一种罕见的角化异常疾病,于 1958 年由弗莱格尔首次描述。HLP 的特征是无症状的小丘疹,对称分布于背部足部和小腿下部。它通常发生在第四或第五个十年。FD 更常见于女性,对这种罕见皮肤病的发病机制知之甚少。组织学检查是确诊所必需的,其特征性表现为:局灶性致密角化过度、变薄的马拉皮氏层、表皮萎缩以及乳头状真皮中密集的带状淋巴细胞浸润。FD 的治疗具有挑战性,目前尚不存在完全有效的标准疗法。我们报告了在意大利米兰的 IRCCS Ca' Granda Ospedale Maggiore Policlinico 皮肤科诊断的 4 例 HLP 病例,并附有文献复习。