Hyperkeratosis Lenticularis Perstans (Flegel Disease)
- Author: Daniel Roling, MD; Chief Editor: William D James, MD more...
Background
Flegel originally described hyperkeratosis lenticularis perstans (HLP) in 1958 as red-brown papules with horny scales of irregular outline measuring 1-5 mm in diameter and up to 1 mm in depth.[1] Lesions are located primarily on the dorsal feet and lower legs, with a decreasing likelihood of manifestation proximally. Most cases have been reported in Europe.
Pathophysiology
No instigating factor has been identified clearly; however, some investigators have implicated UV light. Ultrastructurally, a loss or decreased number of membrane-coating granules (also termed Odland bodies) has been reported. Membrane-coating granules are influential in the normal process of desquamation. A decrease, malformation, or absence of these membrane-coating granules may result in decreased desquamation of the stratum corneum, retention hyperkeratosis, and clinically keratotic hyperkeratosis lenticularis perstans lesions. Some authors have suggested that a cell-mediated cytotoxicity against epithelial cells may be involved in the pathogenesis of hyperkeratosis lenticularis perstans.
Epidemiology
Frequency
United States
No data exist on incidence or prevalence of this disease. Hyperkeratosis lenticularis perstans (Flegel disease) likely has both a familial and nonfamilial variant, since several reports have postulated both an autosomal dominant and a sporadic mode of inheritance.[2]
International
Most cases have been reported in Europe. International incidence is similar to that seen in the United States.
Race
To date, all reports of hyperkeratosis lenticularis perstans have been in white patients.
Sex
No sex predilection is apparent.
Age
Hyperkeratosis lenticularis perstans reportedly occurs most commonly in mid-to-older age groups; however, reports exist of hyperkeratosis lenticularis perstans occurring in patients as young as 13 years.
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