eMedicine Specialties > Dermatology > Papulosquamous Diseases

Hyperkeratosis Lenticularis Perstans (Flegel Disease)

Author: Daniel Roling, MD, Assistant Clinical Professor, Department of Dermatology, Hospital of the University of Pennsylvania
Coauthor(s): Jacqueline M Junkins-Hopkins, MD, Associate Professor, Director, Division of Dermatopathology and Oral Pathology, Department of Dermatology, Johns Hopkins Medical Institutions
Contributor Information and Disclosures

Updated: Feb 20, 2009

Introduction

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.

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.

Clinical

History

Small keratotic papules typically begin to develop symmetrically on the lower extremities.

Clinical photograph of the upper thigh showing nu...

Clinical photograph of the upper thigh showing numerous red-brown papules with sparing of the inguinal crease.

[ CLOSE WINDOW ]
Clinical photograph of the upper thigh showing nu...

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 Medi...

A higher-powered view of the patient seen in Media File 1. Photograph of the upper thigh demonstrates 1- to 4-mm, noncoalescing keratotic papules.

[ CLOSE WINDOW ]
A higher-powered view of the patient seen in Medi...

A higher-powered view of the patient seen in Media File 1. Photograph of the upper thigh demonstrates 1- to 4-mm, noncoalescing keratotic papules.


Papules spread proximally at a slow rate. Hyperkeratosis lenticularis perstans usually is an asymptomatic condition.

Physical

Small, red-brown, hyperkeratotic, 1-5 mm papules on the lower extremities are the most frequent and characteristic presentation of hyperkeratosis lenticularis perstans.3,4 Involvement of the ear pinnae, arms, palms, soles, and the oral mucosa has been reported, although these reports are rare. Involvement of the trunk has been reported but remains an unusual variant.5

Removal of the scale reveals a bright red base, often with pinpoint bleeding. A localized unilateral variant has been reported.6 The trunk tends to be spared; absence of axial lesions is characteristic.

Causes

To date, the causes of hyperkeratosis lenticularis perstans (Flegel disease) are unknown. Some authors suggest that exposure to the sun may be involved.

More on Hyperkeratosis Lenticularis Perstans (Flegel Disease)

Overview: Hyperkeratosis Lenticularis Perstans (Flegel Disease)
Differential Diagnoses & Workup: Hyperkeratosis Lenticularis Perstans (Flegel Disease)
Treatment & Medication: Hyperkeratosis Lenticularis Perstans (Flegel Disease)
Follow-up: Hyperkeratosis Lenticularis Perstans (Flegel Disease)
Multimedia: Hyperkeratosis Lenticularis Perstans (Flegel Disease)
References
[ CLOSE WINDOW ]

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].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

hyperkeratosis lenticularis perstans, Flegel disease, Flegel's disease, HLP, Odland bodies, keratosis, keratotic papules

[ CLOSE WINDOW ]

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.

Medical Editor

Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, 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

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University 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.

Managing Editor

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: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine 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; american college of physicians Honoraria Other

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.