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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The cause of the disease is unknown. There are no systemic manifestions of hyperkeratosis lenticularis perstans. Services on Demand Journal.

Variable reports regarding the vitamin D derivative calcipotriene 0. Clinical findings paired with skin biopsy are diagnostic.

Flegel disease pathology | DermNet NZ

Histopathological differences between early and old lesions of hyperkeratosis Lenticularis Perstans Flegel’s disease. The second, on an old lesion, revealed discrete atrophy and a discrete infiltrate.

Treatment of lesions include:. We believe there was no recurrence because this procedure removed the defective keratinocytes. Treatment with betamethasone, salicylic acid, calcipotriol, tretinoin, cryotherapy and 5-fluorouracil produced little or no effect. CASE REPORT 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 Received on Frenk E, Tapernoux B.

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Actinic keratosis — Actinic dysplasia is seen. The lamellar granules release lipids that are essential to the process of desquamation; without normal desquamation, hyperkeratosis characteristic of this disease develops. An unusual generalized form of hyperkeratosis lenticularis perstans Flegel’s disease. Unfortunately, it is not free to produce. J Am Acad Dermatol ; DermNet NZ does not provide an online consultation service.

Treatment is generally ineffective. Another member of the family was reported by Bean Flegel disease has been reported to rarely affect some families that were also prone to skin and gastrointestinal cancers.

Flegel disease pathology

Multiple asymptomatic hyperkeratotic papules on the lower part of the legs. Pathophysiology There hyperkeratoxis controversial data regarding absent or aberrant lamellar granules ie, Odland perstams, membrane associated granules as seen on electron microscopy in lesional skin contributing to the pathophysiology.

See the DermNet NZ bookstore. How to cite this article. An autosomal dominant skin disease due to lack of a keratinocyte organelle. Curettage of the hyperkeratotic components of the lesion causes pinpoint bleeding. Flegel disease, or hyperkeratosis lenticularis perstans HLP. Mild improvement with treatment noted after 3 weeks Click here to view. Wien Klin Wochenschr ; Disseminated superficial actinic porokeratosis primarily involves the lower legs and sun exposed areas, as well as having the lenticuularis coronoid lamella histologicially.

However, according to some authors, it may be the result of a keratinization disorder and may be triggered by ultraviolet radiation. Lesions are not usually painful. Clinical Synopsis Toggle Dropdown.

Flegel disease

The epidermis under the hyperkeratosis is atrophic compared with the surrounding skin, and hyprkeratosis granular layer is attenuated absent figure 2. Expected results of diagnostic studies Clinical findings paired with skin biopsy are diagnostic. The involvement of the palms and soles is unique to hyperkeratosis lenticularis perstans, and the pathology is distinictive.

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Hyperkeratosis lenticularis perstans HLP Flegel disease. A bonus to all MIMmatch users is the option to sign up for updates on new gene-phenotype relationships. Case report of a rare entity. Approved by the Advisory Board and perstan for publication on While there are reports of improvements with corticosteroids, vitamin D analogs and 5-fluorouracil, there have also been lenticularia of failure to respond to treatment with the first two of these.

J Am Acad of Dermatol. Hyperkeratosis lenticularis perstans HLP is a rare disease first described by Flegel in Hyperkeratosis lenticularis perstans Flegel’s disease – lack of response to treatment with tacalcitol and calcipotriol. The etiology of hyperkeratosis lenticularis perstans is unknown. Hyperkeratosid are usually asymptomatic, but rarely can report mild itching or burning.

Lever’s Histopathology of the skin, 10th ed. Family medical history was unknown because the patient was adopted.

These treatments were not carried out as the patient did not give his consent. Such treatments include CO2 laser, curettage and electrocoagulation.