A collection of disease information resources and questions answered by our Genetic and Rare Diseases Information Specialists for Hyperkeratosis lenticularis . Flegel disease is also known as ‘hyperkeratosis lenticularis perstans’. It was first described by Flegel in It is characterised by red-brown papules with. Hyperkeratosis lenticularis perstans (HLP) is a rare cutaneous disorder occurring in older persons and manifested by multiple benign pink to reddish-brown.

Author: Daikinos Brakora
Country: Lebanon
Language: English (Spanish)
Genre: Finance
Published (Last): 1 February 2011
Pages: 328
PDF File Size: 18.24 Mb
ePub File Size: 5.79 Mb
ISBN: 222-7-78399-878-5
Downloads: 39631
Price: Free* [*Free Regsitration Required]
Uploader: Kazranris

There is controversial data regarding absent or aberrant lamellar granules ie, Odland bodies, membrane associated granules as seen on electron microscopy in lesional skin contributing to the pathophysiology.

Flegel disease pathology | DermNet NZ

Looking For More References? Hyperkeratosis lenticularis perstans HLP is a rare disease first described by Flegel in Another member of the family was reported by Bean The epidermis under the hyperkeratosis is atrophic compared with the surrounding skin, and the granular layer is attenuated absent figure 2. Pathology is characterized by focal hyperkeratosis and parakeratosis. Few lesions of similar morphology on trunk Click here to view.

Hyperkeratosis lenticularis perstans HLP is a rare cutaneous disorder occurring in older persons and manifested by multiple benign pink to reddish-brown keratotic papules that primarily affect the extremities. No sponsor or advertiser has participated in, approved or paid for the content provided by Decision Support in Medicine LLC.

There is a proposed autosomal dominant mode of inheritance, so inquire about a family history.

Flegel disease

Diagnosis is confirmed by histological examination and the clinical evolution of the disease. See the DermNet NZ bookstore. Expert curators review the literature and organize it to facilitate your work. A year-old white male presented with a year-old complaint of asymptomatic reddish papules between 1 and 5 mm in diameter covered with light-colored or brownish scales in the medial and lateral regions of the feet but not on the plantar surfaces or.


Hyperkeratosis Lenticularis Perstans (Flegel’s disease)

Treatment is generally ineffective. We need long-term secure funding to provide you the information that you need at your fingertips. Treatment with betamethasone, salicylic acid, calcipotriol, tretinoin, cryotherapy and 5-fluorouracil produced little or no effect. Two excisional biopsies were performed.

Such treatments include CO2 laser, curettage and electrocoagulation. Expected results of diagnostic studies Clinical findings paired with skin biopsy are diagnostic. Degenerative diseases and perforating disorders. We report a case of hyperkeratosis lenticularis perstans that improved following excisional biopsy of the lesions. Which of the following best describes your perstahs with hand-foot-and-mouth disease?

Lesions are benign and are mainly treated for cosmetic reasons. While there are reports of improvements with corticosteroids, vitamin D analogs and 5-fluorouracil, there have also been reports of failure to respond to treatment with the first two of these.

The histopathological features are hyperorthokeratosis, epidermal atrophy and band-like inflammatory infiltrate in the superficial dermis. Some authors believe Flegel disease is a form of incipient epidermal dysplasia. No laboratory or imaging studies are necessary. The transmission pattern of HLP in the family reported by Bean was consistent lenticulaaris autosomal dominant inheritance.

Hyperkeratosis lenticularis perstans Flegel: Similar lesions on forearms Click here to view. Characteristic findings on physical examination Lesions are multiple, red-brown to grey mm papules with a disc or lens lenticularis shape, sometimes with surrounding erythema. It is characterised by red-brown papules with irregular horny scales located mainly on the top surface of the feet and lower legs.


An autosomal dominant skin disease due to lack of a keratinocyte organelle. Case report of a rare entity.

The favored areas are the lenitcularis feet; however, distal extremities, palms and soles, pinna and oral mucosa can be involved. Stucco keratoses do hyprkeratosis bleed when the scale is peeled back, and will show papillomatosis on pathology. There are no systemic manifestions of hyperkeratosis lenticularis perstans. The mother and 4 children showed the typical hyperkeratotic lesions on the lower leg and dorsum of the foot; however, involvement of the trunk, thigh, arms and dorsum of the hand may occur.

Mild improvement with treatment noted after 3 weeks Click here to view. Multiple asymptomatic hyperkeratotic papules on the lower part of the legs.

While the OMIM database is open to the public, users seeking information about a personal medical or genetic condition are urged to consult with a qualified physician perstns diagnosis and for answers to personal questions. Rare cases report diffuse involvement. Flegel’s disease, also known as hyperkeratosis lenticularis perstans, is a rare skin disease characterized by small reddish-brown asymptomatic hyperkeratotic papules usually located on the lower extremities.

Disseminated superficial actinic porokeratosis primarily involves the lower legs and sun exposed areas, as well as having the pathognomonic coronoid lamella histologicially. Porokeratosis — Cornoid lamellae allow easy diagnosis of this condition.

How to cite this URL:

Author: admin