Hyperkeratosis Lenticularis Perstans (Flegel Disease) Workup

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

Other Tests

Since several reports have associated hyperkeratosis lenticularis perstans (Flegel disease) with an endocrinopathy, obtain a thorough and complete systems review, as well as social and family histories. Laboratory, radiographic, or surgical tests are not needed, unless indicated by information gleaned from these sources.

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Procedures

Skin biopsy with hematoxylin and eosin staining shows characteristic findings of hyperkeratosis lenticularis perstans (Flegel disease). Electron microscopy is not essential.

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Histologic Findings

A discrete area of hyperkeratosis occurs (with areas of parakeratosis) overlying a thinned stratum malpighii and thinned-to-absent granular layer. Irregular acanthosis and some vascular dilatation are peripheral. A lymphoid infiltrate with occasional histiocytes in a bandlike pattern in the papillary dermis typically is seen. See the images below.

Hematoxylin and eosin-stained section, low magnifiHematoxylin 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 magnHematoxylin 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 magnifHematoxylin 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.

Some evidence has indicated that older lesions may show some histologic differences compared with newer ones.[7] Older lesions can show absence of epidermal atrophy and may infiltrate the upper dermis. Ultrastructural studies also reveal the presence of many normal-appearing, membrane-coating granules in the keratinocytes of an old lesion, whereas these normal organelles were not found in the keratinocytes of earlier hyperkeratotic skin lesions.[8]

Electron microscopy

Several authors have reported an absence or decrease in the number of membrane-coating granules, or Odland bodies, within lesional keratinocytes. Although other authors have found Odland bodies to be present, most suggest that the membrane-coating granules may undergo some alterations in number or morphology. Perilesional skin uniformly shows normal keratinocyte differentiation.

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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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