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.
|Published (Last):||17 December 2006|
|PDF File Size:||1.75 Mb|
|ePub File Size:||5.77 Mb|
|Price:||Free* [*Free Regsitration Required]|
Curettage of the hyperkeratotic components of the lesion causes pinpoint bleeding. Disseminated superficial actinic porokeratosis primarily involves the lower legs and sun exposed areas, as well as having the pathognomonic coronoid lamella histologicially. Systemic Lfnticularis and Complications There are no systemic manifestions hyperkeratossi hyperkeratosis lenticularis perstans. It is characterised by red-brown papules with irregular horny scales located mainly on the top surface of the feet and lower legs.
The patient should be made aware of the chronicity of the disease and that the disorder can be difficult to treat. Removal of the scale reveals a bright red base, often with pinpoint bleeding.
In rare instances, the outer ear lobes, arms, palms, soles, and oral mucosa may be affected. Beveridge and Langlands described 2 generations of a kindred manifesting HLP.
None are generally perwtans. The side effect of scarring is a possibility with liquid nitrogen and dermabrasion and an absolute with excision.
No laboratory or imaging studies are necessary. Actinic keratosis — Actinic dysplasia is seen. Hyperkeratosis lenticularis perstans Flegel’s disease – successful treatment with topical corticosteroids.
The histopathological features hyperkdratosis hyperorthokeratosis, epidermal atrophy and band-like inflammatory infiltrate in the superficial dermis.
Lesions are not usually painful. Patient history and clinical examination did not reveal any other diseases.
OMIM Entry – % – HYPERKERATOSIS LENTICULARIS PERSTANS; HLP
Clinical input is often very helpful in making a diagnosis histopathologically. Familial hyperkeratosis lenticularis perstans associat ed with tumours of the skin. Bean reported 3 members of the same family with HLP. What you should be alert for in the history Patients are usually asymptomatic, but rarely can report mild itching or burning.
Hyperkeratosis lenticularis perstans
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. The epidermis under the hyperkeratosis is atrophic compared with the surrounding skin, lenticulzris the granular layer is attenuated absent figure 2.
The disorder is thought to be caused by alterations in the membrane-coating granules lamellar granulesOdland bodies of the keratinocytes. If you have any concerns with your skin or its treatment, see a dermatologist for advice. Flegel’s disease, also known as hyperkeratosis lenticularis perstans, is a rare skin disease characterized oerstans small reddish-brown asymptomatic hyperkeratotic papules usually located on the lower extremities.
Hyperkeratosis lenticularis perstans HLP Flegel disease. Pathophysiology There is controversial data regarding absent or aberrant lamellar granules ie, Lentichlaris bodies, membrane associated granules as seen on electron microscopy in lesional skin contributing to the pathophysiology.
We are determined to keep this website freely accessible.
DermNet NZ does not provide an online consultation service. Odland bodies are round or oval lamellar cytoplasmic organelles found in the upper layers of keratinizing epithelia. Rare cases report diffuse involvement. Services on Demand Journal. How to cite this article. Lesions are benign and are mainly treated for cosmetic reasons.
Hyperkeratosis Lenticularis Perstans (Flegel’s disease)
Frenk and Tapernoux described formation of a compact hyperkeratotic stratum corneum which appeared to be related to absence of Odland bodies in the underlying epidermis. Scraping or peeling off the scale results in pin-point bleeding. The cause pershans the disease is unknown. These treatments were not carried out as the patient did not give his consent. The first, on a new lesion, revealed hyperkeratosis, parakeratosis, epidermal atrophy and dense lymphohistiocytic infiltrate in the upper dermis Figure 2A.
Multiple asymptomatic hyperkeratotic papules on the lower part of the legs. Flegal disease — pathology Kyrle disease Acquired perforating collagenosis Other websites: Flegel disease is a rare dermatosis characterized by asymptomatic hyperkeratotic hyperkeratosiz predominantly on the dorsa of the feet and lower legs.