Stabile Giorgio, Paolino Giovanni, Rizzo Nathalie, Rongioletti Franco
Dermatologia Clinica, Università Vita-Salute San Raffaele, 20132 Milan, Italy.
Dermatologia, IRCCS Ospedale San Raffaele, 20132 Milan, Italy.
Dermatopathology (Basel). 2023 Jan 13;10(1):46-51. doi: 10.3390/dermatopathology10010006.
Hyperkeratosis lenticularis perstans, also known as Flegel's disease (FD), is a rare cutaneous disorder affecting mainly the lower extremities of middle-aged people. Due to its rarity, this disease is usually not recognized by physicians resulting in a delay in diagnosis, especially in those cases with atypical cutaneous involvement. Herein, we present a 72-year-old woman who developed FD characterized by a generalized distribution, involving, in addition to the lower limbs, the trunk and the upper limbs as well. We performed a description of the dermoscopic and pathologic features of this rare entity, also carrying out a brief reappraisal of the cases of FD with a diffuse, atypical and generalized distribution that have been described in the literature. Histopathology with clinical correlation is the cornerstone of the diagnosis, even and especially in atypical cases. This patient with a disease duration of 58 years also represents the longest-lasting case of FD reported in the literature.
持久性豆状角化过度症，也称为弗莱格病（FD），是一种罕见的皮肤疾病，主要影响中年人的下肢。由于其罕见性，这种疾病通常不被医生所认识，导致诊断延迟，尤其是在那些有非典型皮肤受累的病例中。在此，我们报告一名72岁女性，她患有的FD表现为全身性分布，除了下肢外，还累及躯干和上肢。我们描述了这种罕见疾病的皮肤镜和病理特征，还对文献中描述的具有弥漫性、非典型性和全身性分布的FD病例进行了简要重新评估。组织病理学与临床相关性是诊断的基石，即使在非典型病例中也是如此。该患者病程长达58年，也是文献报道中病程最长的FD病例。
