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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J Am Acad Dermatol ; Another reasonable approach is surgical, as it may provide symptomatic and cosmetic results. DermNet NZ does not provide an online consultation service. 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.
It may also be hereditary, with autosomal dominant transmission. Potent topical steroids class I with or without occlusion can be used in a pulsed 4 x daily a week regimen to minimize side effects of atrophy, and can be considered first-line.
The involvement of the palms and soles is unique to hyperkeratosis lenticularis perstans, and the pathology is distinictive. See the DermNet NZ bookstore.
Wolters Kluwer India; The disorder is thought to be caused by alterations in the membrane-coating granules lamellar granulesOdland bodies of the keratinocytes. There is no standard treatment and symptom relief and cosmesis are the primary management goals.
The cause of the disease is unknown. Flegel disease has been reported to rarely affect some families that were also prone to skin and gastrointestinal cancers. These approaches are very much dependent upon the degree of involement.
Flegel disease | DermNet NZ
Hyperkeratosis lenticularis perstans is a rare disorder most commonly affecting middle-aged Caucasians, with a genetic predisposition. The lamellar granules release lipids that are essential to the process of desquamation; without normal desquamation, hyperkeratosis characteristic of this disease develops.
The epidermis under the hyperkeratosis is atrophic compared with lenticularus surrounding skin, and the granular layer is attenuated absent figure 2. 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. All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License. Lesions are not usually painful.
Two excisional biopsies were performed. In Flegel disease, sections show discrete zones of compact lamellar hyperkeratosis with focal parakeratosis figure 1. The genetics of hyperkeratosis lenticularis perstans.
Kyrle disease should exhibit an impressive keratin plug and inflammatory material perforating through the epidermis. Mild improvement with treatment noted after 3 weeks Click here to view. Wien Klin Wochenschr hyperkerwtosis Ultrastructural study of lesional and perilesional skin and therapeutic trial of topical tretinoin versus 5-fluorouracil. 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.
The first, on a new lesion, revealed hyperkeratosis, parakeratosis, epidermal atrophy and dense lymphohistiocytic infiltrate in the upper dermis Figure 2A. Interestingly, however, the lesions that were biopsied healed completely.
Hyperkeratosis Lenticularis Perstans (Flegel’s disease)
Such treatments include CO2 laser, curettage and electrocoagulation. 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.
There hyperkeratosix no recurrence of lesions in the biopsied areas after two years of follow-up. The second, on an old lesion, revealed discrete atrophy and a discrete infiltrate.
Hyperkeratosis lenticularis perstans HLP Flegel disease. The genetics of hyperkeratosis lenticularis perstans. 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.
Kyrle disease — This condition is clinically similar and may share some pathogenic components. No laboratory or imaging studies are necessary. OMIM is intended for use primarily by physicians and other professionals concerned with genetic disorders, by hyperkertosis researchers, and by advanced students in science and medicine. Bean reported 3 members of the same family with HLP.
Flegel disease, or hyperkeratosis lenticularis perstans HLP. The favored areas are the dorsal feet; however, distal extremities, palms and soles, pinna and oral mucosa can be involved. Another member of the lenticuladis was reported by Bean We need long-term secure funding to psrstans you the information that you need at your fingertips.
Patients should be aware that there is no mortality associated with the lesions and understand the risks of each therapeutic modality.
Discrete, follicular papular lesions on dorsa of feet Click here to view. The side effect of scarring is a possibility with liquid nitrogen and dermabrasion and an absolute with excision.
Characteristic findings on physical examination Lesions are multiple, red-brown to grey mm papules with a disc or lenticulais lenticularis shape, sometimes lenticularsi surrounding erythema. The papillary dermis is edematous, contains enlarged vessels, and a lichenoid infiltrate composed of lymphocytes and histiocytes. 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.
A clinicopathological study of Flegel’s disease hyperkeratosis lenticularis perstans. Which of the following best describes your experience with hand-foot-and-mouth disease? 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 What is the Cause of the Disease?