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Hyperkeratosis Lenticularis Perstans (Flegel Disease) Treatment & Management

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

Medical Care

A variety of topical agents have been used to treat this disease, but none is universally effective.

Reports on the effectiveness of tacalcitol and calcipotriol conflict with another report stating they are not effective,[9] and yet another report saying calcipotriol is effective.[10]

Topical 5% fluorouracil and a synthetic vitamin D-3 derivative have been used together with effective results.[10]

The most consistently successful therapies have been the topical application of 5% fluorouracil cream (over several months), local excision, and dermabrasion (see Surgical Care).[11, 12]

In 1986, Gabrielsen reported that hyperkeratosis lenticularis perstans (Flegel disease) was effectively treated with etretinate. Initially, treatment aggravated the condition; however, after 10 weeks of treatment, the papules of hyperkeratosis lenticularis perstans (Flegel disease) nearly all resolved.[13]

Oral retinoids have been successful with continuous therapy. However, short-coarse oral retinoid therapy has also shown success in one case.[14] Patients tend to relapse when therapy is discontinued.

Tretinoin, emollients, psoralen with ultraviolet A (PUVA), and keratolytics have shown varying, but mostly unrewarding, results, even in combination.[15, 16, 17] Topical steroids have similarly shown mixed results, although betamethasone dipropionate showed a response in a 2008 case study.[18]

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Surgical Care

Dermabrasion is a possible surgical modality. However, a large number of lesions, as well as lesion location, make this an impractical approach. Local excision may be successful, especially if the number of lesions is small. Cryotherapy is an additional possibility.

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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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