Hyperkeratosis lenticularis perstansL85.8

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 02.03.2022

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Synonym(s)

flail disease; Flegel's disease; Hyperkeratosis lenticularis perstans flail; lout's disease

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HistoryThis section has been translated automatically.

Boor, 1958

DefinitionThis section has been translated automatically.

Rare, probably autosomal dominant inherited androtropic genodermatosis with disseminated small papular keratoses on the lower legs persisting chronically over years. The term "lenticularis" refers to the lenticular-sized (or even larger), flat whitish plaques.

EtiopathogenesisThis section has been translated automatically.

Unknown, most likely autosomal dominant inheritance; also sporadic; probably a disorder of keratinocyte proliferation plays a role. Associations with primary hyperaldosteronism have been described (Zhang X et al. 2018).

ManifestationThis section has been translated automatically.

In the second half of life, m>w.

LocalizationThis section has been translated automatically.

Beginning on the back of the foot in middle age, later spreading to the extensor sides of the lower legs and possibly including the thighs, arms, back of the hands and rarely the trunk (here also described as a unilateral cutaneous mosaic). After detachment of the firmly adhering horny scales, punctiform bleeding appears at the base. Also soles of the feet and palms may be involved; here appearing as small horny papules (pits).

Clinical featuresThis section has been translated automatically.

Disseminated, symmetrical, asymptomatic, up to 0.5 cm large, centrally sunken, brown-red or even whitish, hyperkeratotic papules with firmly adherent scaling. Dewdrop-like bleeding after removal of the scale. Confluence results in small psoriasiform foci. Unilateral distribution patterns have also been described (Urbina F et al. 2016).

HistologyThis section has been translated automatically.

Bowl-shaped depressed surface relief. Here, compact orthohyperkeratosis, sometimes also parahyperkeratosis with uniform epidermal atrophy. Lesional, band-like (but not epitheliotropic), lymphocytic infiltrate in the upper dermis; absence of elastic fibers. Laterally, the epithelium is acanthotic and encompasses the central horny papule in a collerette-like fashion.

Electron microscopy: absence of Odland bodies in the keratinocytes.

Differential diagnosisThis section has been translated automatically.

Clinical:

Histologic:

External therapyThis section has been translated automatically.

Treatment trial with 5% 5-fluorouracil cream(Efudix) under occlusion 2 times/day for several weeks under stationary conditions.

Alternatively: glucocorticoid externa.

In addition to this, nurturing (urea-containing) external agents such as Ungt. emulsif. aq. and 5% urea cream R102.

Internal therapyThis section has been translated automatically.

Acitretin in low dosage (5-10 mg/day) leads to success in some cases.

LiteratureThis section has been translated automatically.

  1. Blaheta HJ et al (2001) Hyperkeratosis lenticularis perstans (Flegel's disease)--lack of response to treatment with tacalcitol and calcipotriol. Dermatology 202: 255-258
  2. Bortoluzzi P et al (2021) Hyperkeratosis lenticularis perstans (Flegel's disease): our experience and review of the literature. Int J Dermatol 60: 33-38.
  3. Flegel H (1958) Hyperkeratosis lenticularis perstans. Dermatologist 9: 362-364
  4. Krishnan A et al. (2012) Photoletter to the editor: Hyperkeratosis lenticularis perstans (Flegel's disease) with unusual clinical presentation. Response to isotretinoin therapy. J Dermatol Case Rep 6:93-95.
  5. Langer K et al (1992) Hyperkeratosis lenticularis perstans (Flegel's disease) J Am Acad Dermatol 27: 812-6.
  6. Piotrowski MJ et al (2015) JAAD Grand Rounds quiz: Red-brown hyperkeratotic papules in a 69-year-old man. J Am Acad Dermatol. 73:346-348
  7. Price ML, Wilson Jones E, MacDonald DM (1987) A clinicopathological study of Flegel's disease (hyperkeratosis lenticularis perstans). Br J Dermatol 116: 681-691
  8. Ravat FE et al (2003) Case 3: widespread scaly papules affecting a patient's limbs and pinnae giving the skin a rough feeling. Diagnosis: Flegel's disease. Syn. hyperkeratosis lenticularis perstans. Clin Exp Dermatol 28: 339-340.
  9. Wilson PD et al (1980) Treatment of hyperkeratosis lenticularis perstans (Flegel) with topical fluorouracil. Dermatologica 160: 337-340
  10. Urbina F et al (2016) A case of localized, unilateral hyperkeratosis lenticularis perstans on a woman's breast. J Dtsch Dermatol Ges 14:416-41.
  11. Zhang X et al (2018) Hyperkeratosis lenticularis perstans in a patient with primary hyperaldosteronism. J Dtsch Dermatol Ges 16: 72-73.
  12. Zimmermann R (2001) 40 years to Flegel's disease (hyperkeratosis lenticularis perstans). Dermatol 52: 231-235

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Last updated on: 02.03.2022