Widespread and Long-Enduring Hyperkeratosis Lenticularis Perstans (Flegel’s Disease): Clinico-Pathological and Dermoscopic Features of a Rare Presentation

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 w...

Full description

Bibliographic Details
Main Authors: Giorgio Stabile, Giovanni Paolino, Nathalie Rizzo, Franco Rongioletti
Format: Article
Language:English
Published: MDPI AG 2023-01-01
Series:Dermatopathology
Subjects:
Online Access:https://www.mdpi.com/2296-3529/10/1/6
_version_ 1797612447022972928
author Giorgio Stabile
Giovanni Paolino
Nathalie Rizzo
Franco Rongioletti
author_facet Giorgio Stabile
Giovanni Paolino
Nathalie Rizzo
Franco Rongioletti
author_sort Giorgio Stabile
collection DOAJ
description 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.
first_indexed 2024-03-11T06:42:20Z
format Article
id doaj.art-f16f7c3e0b2e46bb8f1570d344dbaffb
institution Directory Open Access Journal
issn 2296-3529
language English
last_indexed 2024-03-11T06:42:20Z
publishDate 2023-01-01
publisher MDPI AG
record_format Article
series Dermatopathology
spelling doaj.art-f16f7c3e0b2e46bb8f1570d344dbaffb2023-11-17T10:32:41ZengMDPI AGDermatopathology2296-35292023-01-01101465110.3390/dermatopathology10010006Widespread and Long-Enduring Hyperkeratosis Lenticularis Perstans (Flegel’s Disease): Clinico-Pathological and Dermoscopic Features of a Rare PresentationGiorgio Stabile0Giovanni Paolino1Nathalie Rizzo2Franco Rongioletti3Dermatologia Clinica, Università Vita-Salute San Raffaele, 20132 Milan, ItalyDermatologia Clinica, Università Vita-Salute San Raffaele, 20132 Milan, ItalySurgical Pathology, IRCCS Ospedale San Raffaele, 20132 Milan, ItalyDermatologia Clinica, Università Vita-Salute San Raffaele, 20132 Milan, ItalyHyperkeratosis 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.https://www.mdpi.com/2296-3529/10/1/6hyperkeratosis lenticularis perstansFlegel’s diseasehistopathologydermoscopy
spellingShingle Giorgio Stabile
Giovanni Paolino
Nathalie Rizzo
Franco Rongioletti
Widespread and Long-Enduring Hyperkeratosis Lenticularis Perstans (Flegel’s Disease): Clinico-Pathological and Dermoscopic Features of a Rare Presentation
Dermatopathology
hyperkeratosis lenticularis perstans
Flegel’s disease
histopathology
dermoscopy
title Widespread and Long-Enduring Hyperkeratosis Lenticularis Perstans (Flegel’s Disease): Clinico-Pathological and Dermoscopic Features of a Rare Presentation
title_full Widespread and Long-Enduring Hyperkeratosis Lenticularis Perstans (Flegel’s Disease): Clinico-Pathological and Dermoscopic Features of a Rare Presentation
title_fullStr Widespread and Long-Enduring Hyperkeratosis Lenticularis Perstans (Flegel’s Disease): Clinico-Pathological and Dermoscopic Features of a Rare Presentation
title_full_unstemmed Widespread and Long-Enduring Hyperkeratosis Lenticularis Perstans (Flegel’s Disease): Clinico-Pathological and Dermoscopic Features of a Rare Presentation
title_short Widespread and Long-Enduring Hyperkeratosis Lenticularis Perstans (Flegel’s Disease): Clinico-Pathological and Dermoscopic Features of a Rare Presentation
title_sort widespread and long enduring hyperkeratosis lenticularis perstans flegel s disease clinico pathological and dermoscopic features of a rare presentation
topic hyperkeratosis lenticularis perstans
Flegel’s disease
histopathology
dermoscopy
url https://www.mdpi.com/2296-3529/10/1/6
work_keys_str_mv AT giorgiostabile widespreadandlongenduringhyperkeratosislenticularisperstansflegelsdiseaseclinicopathologicalanddermoscopicfeaturesofararepresentation
AT giovannipaolino widespreadandlongenduringhyperkeratosislenticularisperstansflegelsdiseaseclinicopathologicalanddermoscopicfeaturesofararepresentation
AT nathalierizzo widespreadandlongenduringhyperkeratosislenticularisperstansflegelsdiseaseclinicopathologicalanddermoscopicfeaturesofararepresentation
AT francorongioletti widespreadandlongenduringhyperkeratosislenticularisperstansflegelsdiseaseclinicopathologicalanddermoscopicfeaturesofararepresentation