Hyperkeratosis Lenticularis Perstans (Flegel Disease) 

  • Author: Daniel Roling, MD; Chief Editor: William D James, MD   more...
 
Updated: Jul 11, 2011
 

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.

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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.

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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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Contributor Information and Disclosures
Author

Daniel Roling, MD  Assistant Clinical Professor, Department of Dermatology, Hospital of the University of Pennsylvania

Daniel Roling, MD is a member of the following medical societies: American Academy of Dermatology and Pennsylvania Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

Jacqueline M Junkins-Hopkins, MD  Associate Professor, Director, Division of Dermatopathology and Oral Pathology, Department of Dermatology, Johns Hopkins Medical Institutions

Jacqueline M Junkins-Hopkins, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, and American Society of Dermatopathology

Disclosure: Nothing to disclose.

Specialty Editor Board

Noah S Scheinfeld, MD, JD, FAAD  Assistant Clinical Professor, Department of Dermatology, Columbia University College of Physicians and Surgeons; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, and New York Eye and Ear Infirmary; Private Practice

Noah S Scheinfeld, MD, JD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Optigenex Consulting fee Independent contractor

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD  Herman Beerman Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System

Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology and American Society of Dermatopathology

Disclosure: Lippincott Williams Wilkins Royalty Textbook editor; DLA Piper Consulting fee Consulting

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD  Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System

William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: Elsevier Royalty Other

References

  1. Flegel H. [Hyperkeratosis lenticularis perstans.]. Hautarzt. Aug 1958;9(8):363-4. [Medline].

  2. Bean SF. The genetics of hyperkeratosis lenticularis perstans. Arch Dermatol. Jul 1972;106(1):72. [Medline].

  3. Humphrey S, Crawford RI, Au S. Long-standing spiny papules on the lower extremities. Flegel disease, or hyperkeratosis lenticularis perstans (HLP). Arch Dermatol. Nov 2008;144(11):1509-14. [Medline].

  4. Li TH, Hsu CK, Chiu HC, Chang CH. Multiple asymptomatic hyperkeratotic papules on the lower part of the legs. Hyperkeratosis lenticularis perstans (HLP) (Flegel disease). Arch Dermatol. Jul 1997;133(7):910-1, 913-4. [Medline].

  5. Miljkovic J. An unusual generalized form of hyperkeratosis lenticularis perstans (Flegel's disease). Wien Klin Wochenschr. 2004;116 Suppl 2:78-80. [Medline].

  6. Miranda-Romero A, Sanchez Sambucety P, Bajo del Pozo C, Martinez Fermandez M, Esquivias Gomez JI, Garcia Munoz M. Unilateral hyperkeratosis lenticularis perstans (Flegel's disease). J Am Acad Dermatol. Oct 1998;39(4 Pt 1):655-7. [Medline].

  7. Ando K, Hattori H, Yamauchi Y. Histopathological differences between early and old lesions of hyperkeratosis Lenticularis Perstans (Flegel's disease). Am J Dermatopathol. Apr 2006;28(2):122-6. [Medline].

  8. Jang KA, Choi JH, Sung KJ, Moon KC, Koh JK. Hyperkeratosis lenticularis perstans (Flegel's disease): histologic, immunohistochemical, and ultrastructural features in a case. Am J Dermatopathol. Aug 1999;21(4):395-8. [Medline].

  9. Blaheta HJ, Metzler G, Rassner G, Garbe C. Hyperkeratosis lenticularis perstans (Flegel's disease)--lack of response to treatment with tacalcitol and calcipotriol. Dermatology. 2001;202(3):255-8. [Medline].

  10. Metze D, Lubke D, Luger T. [Hyperkeratosis lenticularis perstans (Flegel's disease) - a complex disorder of epidermal differentiation with good response to a synthetic vitamin D3 derivate]. Hautarzt. Jan 2000;51(1):31-5. [Medline].

  11. Pearson LH, Smith JG Jr, Chalker DK. Hyperkeratosis lenticularis perstans (Flegel's disease). Case report and literature review. J Am Acad Dermatol. Jan 1987;16(1 Pt 2):190-5. [Medline].

  12. Wilson PD, Ive FA. Treatment of hyperkeratosis lenticularis perstans (Flegel) with topical fluorouracil. Dermatologica. 1980;160(5):337-40. [Medline].

  13. Gabrielsen TO. [Tigason in hyperkeratosis lenticularis perstans (HLP)--a case report]. Z Hautkr. Feb 15 1986;61(4):214-7. [Medline].

  14. Cooper SM, George S. Flegel's disease treated with psoralen ultraviolet A. Br J Dermatol. Feb 2000;142(2):340-2. [Medline].

  15. Langer K, Zonzits E, Konrad K. Hyperkeratosis lenticularis perstans (Flegel's disease). Ultrastructural study of lesional and perilesional skin and therapeutic trial of topical tretinoin versus 5-fluorouracil. J Am Acad Dermatol. Nov 1992;27(5 Pt 2):812-6. [Medline].

  16. Lindsay E. Zinc paste bandages and the treatment of Flegel's disease. Br J Community Nurs. Mar 2005;10(3):S14-9. [Medline].

  17. Sterneberg-Vos H, van Marion AM, Frank J, Poblete-Gutierrez P. Hyperkeratosis lenticularis perstans (Flegel's disease) - successful treatment with topical corticosteroids. Int J Dermatol. Nov 2008;47 Suppl 1:38-41. [Medline].

  18. Ishibashi A, Tsuboi R, Fujita K. Familial hyperkeratosis lenticularis perstans. Associated with cancers of the digestive organs. J Dermatol. Aug 1984;11(4):407-9. [Medline].

  19. Ralfs IG, Ryan TJ, Dawber RP. Hyperkeratosis lenticularis perstans (Flegel's disease). Br J Dermatol. 1980;103:48.

  20. Tidman MJ, Price ML, MacDonald DM. A histopathological and ultrastructural study of hyperkeratosis lenticularis perstans. Clin Exp Dermatol. 1987;12:231.

  21. van de Staak WJ, Bergers AM, Bongaarts P. Hyperkeratosis lenticularis perstans (Flegel). Dermatologica. 1980;161(5):340-6. [Medline].

 
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Clinical photograph of the upper thigh showing numerous red-brown papules with sparing of the inguinal crease.
A higher-powered view of the patient seen in the previous image. Photograph of the upper thigh demonstrates 1- to 4-mm, noncoalescing keratotic papules.
Hematoxylin and eosin-stained section, low magnification. Epidermal hyperplasia with rete elongation surmounted by a thickened, compact, hyperkeratotic scale. A bandlike lymphoid infiltrate expands the papillary dermis.
Hematoxylin and eosin-stained section, medium magnification. The lateral edge of the lesion demonstrates abrupt hyperkeratosis and a combination of epidermal atrophy and acanthosis.
Hematoxylin and eosin-stained section, high magnification. The section shows mostly orthokeratotic scale, thinning of the epidermis with a diminished granular cell layer, and an infiltrate of lymphocytes in the superficial dermis, which approximate the dermal-epidermal interface.
 
 
 
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