Pathogenic variants in the SPTLC1 gene cause hyperkeratosis lenticularis perstans.

Background: Hyperkeratosis lenticularis perstans (HLP), also known as Flegel disease, is a rare skin disease presenting with asymptomatic small hyperkeratotic papules. The lesions often appear on the dorsal feet and lower legs, and typically develop after the fourth decade of life. A genetic basis for HLP is suspected; however, so far no gene defect linked to the development of HLP has been identified.

Widespread and Long-Enduring Hyperkeratosis Lenticularis Perstans (Flegel's Disease): Clinico-Pathological and Dermoscopic Features of a Rare Presentation.

Hyperkeratosis lenticularis perstans, also known as Flegel's disease (FD), is a rare cutaneous disorder affecting mainly the lower extremities of middle-aged people. Due to its rarity, this disease is usually not recognized by physicians resulting in a delay in diagnosis, especially in those cases with atypical cutaneous involvement. Herein, we present a 72-year-old woman who developed FD characterized by a generalized distribution, involving, in addition to the lower limbs, the trunk and the upper limbs as well.

Hyperkeratosis lenticularis perstans (Flegel's disease): our experience and review of the literature.

Hyperkeratosis lenticularis perstans (HLP), or Flegel's disease (FD), is a rare disorder of keratinization first described in 1958 by Flegel. HLP is characterized by asymptomatic small papules distributed symmetrically on the dorsal feet and lower part of the legs. It typically presents in the fourth or fifth decade of life.

Detection of Borrelia burgdorferi sensu stricto and Borrelia garinii DNAs in patient with Hyperkeratosis lenticularis perstans (Flegel disease).

Determination of the causative agent of erythema-like skin lesions in case of nonspecific superficial perivascular dermatitis was supported by histological examination and led to the latter diagnosis of Hyperkeratosis lenticularis perstans (Flegel disease) in patient. The presence of antibodies against Borrelia burgdorferi in patient serum was confirmed by a routine ELISA method and verified by Western blot technique. Skin biopsy and blood specimens were analyzed by PCR and multilocus sequence analysis (MLSA).

Photoletter to the editor: Hyperkeratosis lenticularis perstans (Flegel's disease) with unusual clinical presentation. Response to isotretinoin therapy.

Hyperkeratosis lenticularis perstans also known as Flegel's disease is a keratinisation disorder characterized by small keratotic papules with horny scales. Most cases have been reported in Europe with age of presentation between 35 and 60 years. We report a case of a 25-year-old man, who presented with 1-5 mm multiple asymptomatic hyperkeratotic papules of 15 years duration on both legs and hand along with lichenified plaques with Koebner phenomenon in the axillary folds, anticubital and popliteal fossae.

Hyperkeratosis lenticularis perstans.

Flegel's disease, also known as hyperkeratosis lenticularis perstans, is a rare skin disease characterized by small reddish-brown asymptomatic hyperkeratotic papules usually located on the lower extremities. The histopathological features are hyperorthokeratosis, epidermal atrophy and band-like inflammatory infiltrate in the superficial dermis. Treatment is generally ineffective.

Morphological evidence of periodical exacerbation of hyperkeratosis lenticularis perstans.

We present a case of hyperkeratosis lenticularis perstans (Flegel's disease) in a 71-year-old-woman. Apart from all the typical morphological features of the disease, we evidenced mounds of parakeratosis that contained neutrophils, alternating with other strata of the horny layers in which no infiltrate was seen. Such a pattern has been described in association with diseases that show a periodic clinical pattern of presentation with periods of exacerbations and remissions.