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Hyperkeratosis Lenticularis Perstans (Flegel Disease) Medication

  • Author: Daniel Roling, MD; Chief Editor: William D James, MD  more...
 
Updated: Aug 31, 2015
 

Medication Summary

These medications have proven the most efficacious for the treatment of hyperkeratosis lenticularis perstans (Flegel disease).

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Topical cytotoxic agents

Class Summary

Inhibit cell growth and proliferation.

Fluorouracil topical (Efudex, Fluoroplex)

 

Fluorouracil has been in clinical use since the 1960s. Systemic absorption is limited to approximately 6% of the applied dose and is selectively higher in abnormal skin. Fluorouracil interferes with pyrimidine metabolism by inhibiting thymidylate synthetase, thus inhibiting DNA synthesis.

It is available as 5% (Efudex) or 1% (Fluoroplex) cream, 2% or 5% solution (both Efudex), or 1% solution (Fluoroplex).

If applied with the fingers, wash hands immediately after application.

Fluorouracil is FDA approved for the treatment of actinic keratosis and superficial basal cell carcinomas.

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Vitamins

Class Summary

Essential for normal DNA synthesis.

Calcipotriene (Dovonex)

 

Calcipotriene is a  synthetic vitamin D-3 derivative that possesses therapeutic properties similar to vitamin D-3, including inhibition of epidermal proliferation, induction of differentiation, and anti-inflammatory effects.

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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, 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, American Society of Dermatopathology

Disclosure: Nothing to disclose.

Specialty Editor Board

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, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD Herman Beerman 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, American Medical Association, American Society of Dermatopathology, Pennsylvania Academy of Dermatology

Disclosure: Received royalty from Lippincott Williams Wilkins for textbook editor.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

Noah S Scheinfeld, JD, MD, FAAD Assistant Clinical Professor, Department of Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private Practice

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

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Abbvie<br/>Received income in an amount equal to or greater than $250 from: Optigenex<br/>Received salary from Optigenex for employment.

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

  2. Bean SF. The genetics of hyperkeratosis lenticularis perstans. Arch Dermatol. 1972 Jul. 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. 2008 Nov. 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. 1997 Jul. 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. 1998 Oct. 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. 2006 Apr. 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. 1999 Aug. 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. 2000 Jan. 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. 1987 Jan. 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. 1986 Feb 15. 61(4):214-7. [Medline].

  14. Krishnan A, Kar S. Photoletter to the editor: Hyperkeratosis lenticularis perstans (Flegel's disease) with unusual clinical presentation. Response to isotretinoin therapy. J Dermatol Case Rep. 2012 Sep 28. 6(3):93-5. [Medline]. [Full Text].

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

  16. 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. 1992 Nov. 27(5 Pt 2):812-6. [Medline].

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

  18. 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. 2008 Nov. 47 Suppl 1:38-41. [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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