HYPERKERATOSIS LENTICULARIS PERSTANS PDF
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.
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Some authors believe Flegel disease is a form of incipient epidermal dysplasia. OMIM is intended for use primarily by physicians and other professionals concerned with genetic hyperkeeatosis, by genetics researchers, and by advanced students in science and medicine.
Pink or reddish-brown scaly papules from 1 to 5 mm in size developed in the third hyperkeatosis fourth hperkeratosis of life. Hyperkeratosis lenticularis perstans is a rare disorder most commonly affecting middle-aged Caucasians, with a genetic predisposition. No laboratory or imaging studies are necessary. Clinical Synopsis Toggle Dropdown.
Variable reports regarding the vitamin D derivative calcipotriene 0. The disorder was first described by Flegel summary by Bean, There is no standard treatment and symptom relief and cosmesis are the primary management goals. In Flegel disease, sections show discrete zones of compact lamellar hyperkeratosis with focal parakeratosis figure 1.
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Hyperkeratosis lenticularis perstans
There is a proposed autosomal dominant mode of inheritance, so inquire about a family history. Flegel disease has been reported to rarely affect some families that were also prone to skin and gastrointestinal cancers.
Diagnosis is confirmed by histological examination and the clinical evolution of the disease. Similar lesions on forearms Click here to view. Self-skin examination New smartphone apps to check your skin Learn more Sponsored content. There was no recurrence of lesions in the biopsied areas after two years of follow-up. You must be a registered member of Dermatology Advisor to post a comment. Lever’s Histopathology of the skin, 10th ed.
The epidermis under the hyperkeratosis is atrophic compared with the surrounding skin, and the granular layer is attenuated absent figure 2. Search Pubmed for Patel KB.
Flegel disease pathology
If lesions are diffuse surgical options would hyperkeratisis unreasonable. Approved by the Advisory Board and accepted for publication on Beveridge and Langlands described 2 generations of a kindred manifesting HLP.
Rare cases report diffuse involvement. Dermatopathology glossary Flegel disease Dermatopathology index Books about skin diseases: The papillary dermis is edematous, contains enlarged vessels, and a lichenoid infiltrate composed of lymphocytes and histiocytes. It was first described by Flegel in Familial hyperkeratosis lenticularis perstans.
Flegel disease | DermNet NZ
What is the Cause of the Disease? There are rare reports of association with endocrine diseases, such as diabetes and hypothroidism.
Associated with lenticulxris of the digestive organs. We believe there was no recurrence because this procedure removed the defective keratinocytes.
Treatment is generally ineffective. Lesions on thighs Click here to view. The genetics of hyperkeratosis lenticularis perstans. The cause of the disease is perstana. The disorder is thought to be caused by alterations in the membrane-coating granules lamellar granulesOdland bodies of the keratinocytes. Such treatments include CO2 laser, curettage and electrocoagulation.