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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Etiology The etiology of hyperkeratosis lenticularis perstans is unknown. Treatment Options Treatment options are summarized in Table I. Curettage of the hyperkeratotic components of the lesion causes pinpoint bleeding. Cooper, S, George, S. Pathology is characterized by focal hyperkeratosis and parakeratosis.

Hyperkeratosis Lenticularis Perstans (Flegel’s disease)

A clinicopathological study of Flegel’s disease hypedkeratosis lenticularis perstans. Topical retinoids at the highest strength tolerable have been used. 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.

This family had a high incidence of skin tumors, including squamous and basal cell carcinomas, in areas other than those affected by the hyperkeratotic lesions. 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.

Disseminated superficial actinic porokeratosis primarily involves the lower legs and sun exposed areas, as well as having the pathognomonic coronoid lamella histologicially. Treatment is generally ineffective. Search Pubmed for Patel KB. Hyperkeratosis lenticularis perstans Flegel. Multiple asymptomatic hyperkeratotic papules on the lower part of the legs.

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Flegel disease pathology | DermNet NZ

Odland bodies are round or oval lamellar cytoplasmic organelles found in the upper layers of keratinizing epithelia. Pathophysiology 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.

Family medical history was unknown because the patient was adopted. An unusual generalized form of hyperkeratosis lenticularis perstans Flegel’s disease. Lesions are multiple, red-brown to grey mm papules with a disc or lens lenticularis shape, sometimes with surrounding erythema.

Their function is not known. The disorder was first described by Flegel summary by Bean, hypwrkeratosis Lesions are small, red-brown, mm scaly papules that appear most commonly on the top surface of the feet and lower legs.

The patient should be made aware of the chronicity of the lenticukaris and that the disorder can be difficult to treat. Hyperkeratosis lenticularis perstans HLP is a rare disease first described by Flegel in Hyperkeratosis lenticularis perstans is a rare disorder most commonly affecting middle-aged Caucasians, with a genetic predisposition. See the DermNet NZ bookstore. A bonus to all MIMmatch users is the option to sign up for updates on new gene-phenotype relationships.

It may also be hereditary, with autosomal dominant transmission. Beveridge and Langlands described 2 generations of a kindred manifesting HLP.

If lesions are diffuse surgical options would be unreasonable. Some authors believe Flegel disease is a form of incipient epidermal dysplasia. Expert curators review the literature and organize it to facilitate your work.

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Flegel disease

Another reasonable perstahs is surgical, as it may provide symptomatic and cosmetic results. There was no recurrence of lesions in the biopsied areas after two years of follow-up. Examination of the membrane-coating granules by electon microscopy is commonly reported in the literature but is not considered useful for the diagnosis. The epidermis under the hyperkeratosis is atrophic compared with the surrounding skin, and the granular layer is attenuated absent figure 2.

Lesions on thighs Click here to view. The cause of the disease is unknown.

Hyperkeratosis Lenticularis Perstans (Flegel’s disease)

Histologically, the condition is characterized by hyperkeratosis, a thinning or absence of the granular layer, epidermal atrophy and a band-like infiltrate in the upper dermis. Clinical input is often very helpful in making a diagnosis histopathologically.

J Am Acad Dermatol ; Lesions are benign and are mainly treated for cosmetic reasons. Patients should be aware that there is no mortality associated with the lesions and understand the risks of each therapeutic modality.

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 for diagnosis and for answers to personal questions.