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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Patients are usually asymptomatic, but rarely can report mild itching or burning. There is a proposed autosomal dominant mode of inheritance, so inquire about a family hypetkeratosis.
Lesions are multiple, red-brown to grey mm papules with a disc or lens lenticularis shape, sometimes with surrounding erythema. The favored areas are the dorsal feet; however, distal extremities, palms and soles, pinna and oral mucosa can be involved. Rare lentciularis report diffuse involvement. Scraping or peeling off the scale results in pin-point bleeding. Clinical findings paired with skin biopsy are diagnostic. Pathology is characterized by focal hyperkeratosis and parakeratosis. The epidermis is atrophic and shows hypogranulosis as well as a thinned spinous layer.
The papillary dermis is edematous, contains enlarged vessels, and a lichenoid lentlcularis composed of lymphocytes and histiocytes. No laboratory or imaging studies are necessary. The involvement of the lenticularie and soles is unique to hyperkeratosis lenticularis perstans, and the pathology is distinictive.
Stucco keratoses do not bleed when the scale is peeled back, and will show papillomatosis hyperkratosis pathology. Disseminated superficial actinic porokeratosis primarily involves the lower legs and sun exposed areas, as well as having the pathognomonic coronoid lamella histologicially. Hyperkeratosis lenticularis perstans is a rare disorder most commonly affecting middle-aged Caucasians, with a genetic predisposition.
There is controversial data regarding absent or aberrant lamellar granules ie, Odland bodies, membrane associated granules as seen on electron microscopy in lenticluaris skin contributing to the pathophysiology. The lamellar granules release lipids that are essential to the process of desquamation; without normal desquamation, hyperkeratosis characteristic of this disease develops. There are no systemic manifestions of hyperkeratosis lenticularis perstans.
There are rare reports of association with endocrine diseases, such as diabetes and hypothroidism.
Flegel disease pathology
Complications are primarily cosmetic, as lesions are usually multiple and tend to spread proximally. The lesions of hyperkeratosis lenticularis perstans are usually asymptomatic but cosmetically frustrating.
Reports have noted that older lesions are more resistant to treatment than more recent lesions. Topical treatments are a reasonable first approach. 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.
Topical retinoids at the highest strength tolerable have been used. Variable reports regarding the vitamin D derivative calcipotriene 0. Systemic retinoids have been reported, but justifying their harmful side effects perstnas treat a benign asymptomatic condition is difficult. Another reasonable approach is surgical, as it may provide symptomatic and cosmetic results. These approaches are very much dependent upon the degree of involement.
If lesions are diffuse surgical options would be unreasonable. The side effect of scarring is a possibility with liquid nitrogen and dermabrasion and an absolute with excision. Finally, short-term psoralen with ultraviolet radiation has been used in a normal psoriasis regimen.
The lenticulariz clear with relatively few treatments, however relapse gradually occurs. It is again difficult to justify the potential harmful side effects of this treatment for a benign condition.
The patient should be made aware of the chronicity of the disease and that the disorder can hyperkeratodis difficult to treat. There is no standard treatment and symptom relief and cosmesis are the primary management goals. Patients should be aware that there is no mortality associated with the lesions and understand the risks of each therapeutic modality.
J Am Acad of Dermatol. Cooper, S, George, S.
J Am Acad Dermatol. No sponsor or advertiser has participated in, approved or paid for the content provided by Decision Support in Medicine LLC.
Flegel disease pathology | DermNet NZ
Who is at Risk for Developing this Disease? What is the Cause of the Disease? What you should be alert for in the history Patients hypetkeratosis usually asymptomatic, but rarely can report mild itching or burning.
Characteristic findings on physical examination Lesions are multiple, red-brown to grey mm papules with a disc or lenhicularis lenticularis shape, sometimes with surrounding erythema. Expected results of diagnostic studies Clinical findings paired with skin biopsy are diagnostic. Etiology The etiology of hyperkeratosis lenticularis perstans is unknown.
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. Systemic Implications and Complications There are no systemic manifestions of hyperkeratosis lenticularis perstans.
Treatment Options Treatment options are summarized in Table I. Powered By Decision Lejticularis in Medicine. You must be a registered member of Dermatology Advisor to post a comment.