Hyperkeratosis lenticularis perstans (Flegel's disease): our experience and review of the literature.

Flegel's disease hyperkeratosis lenticularis perstans

Journal

International journal of dermatology
ISSN: 1365-4632
Titre abrégé: Int J Dermatol
Pays: England
ID NLM: 0243704

Informations de publication

Date de publication:
Jan 2021
Historique:
received: 28 10 2019
revised: 14 04 2020
accepted: 16 04 2020
pubmed: 14 5 2020
medline: 6 1 2021
entrez: 14 5 2020
Statut: ppublish

Résumé

Hyperkeratosis lenticularis perstans (HLP), or Flegel's disease (FD), is a rare disorder of keratinization first described in 1958 by Flegel. HLP is characterized by asymptomatic small papules distributed symmetrically on the dorsal feet and lower part of the legs. It typically presents in the fourth or fifth decade of life. FD has been reported more commonly in women, and little is known about the pathogenesis of this rare dermatosis. Histological examination is mandatory to confirm the diagnosis, and the characteristic features are: focal compact hyperkeratosis, thinned stratum malpighii, epidermal atrophy, and a dense, band-like lymphocytic infiltrate in the papillary dermis. The treatment of FD is challenging, and a fully effective standard therapy does not exist. We report four cases of HLP that have been diagnosed at the Dermatology Unit of IRCCS Ca' Granda Ospedale Maggiore Policlinico in Milan, Italy, and a literature review is enclosed.

Identifiants

pubmed: 32403156
doi: 10.1111/ijd.14930
doi:

Types de publication

Case Reports Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

33-38

Informations de copyright

© 2020 the International Society of Dermatology.

Références

Flegel H. Hyperkeratosis lenticularis perstans. Hautarzt 1958; 9(8): 363-364.
Krishnan A, Kar S. Photoletter to the editor: Hyperkeratosis lenticularis perstans (Flegel's disease) with unusual clinical presentation. Response isotretinoin therapy. J Dermatol Case Rep 2012; 6(3): 93-95.
Fernández-Crehuet P, Rodríguez-Rey E, Ríos-Martín JJ, Camacho FM. Hyperkeratosis lenticularis perstans, or Flegel disease, with palmoplantar involvement. Actas Dermosifiliogr 2009; 100(2): 157-159.
Miranda-Romero A, Sanchez Sambucety P, del Pozo CB. Unilateral hyperkeratosis lenticularis perstans (Flegel's disease). J Am Acad Dermatol 1998; 39(4): 655-657.
Jang KA, Choi JH, Sung KJ, Moon KC, Koh J-K. Hyperkeratosis lenticularis perstans (Flegel's disease): Histologic, immunohistochemical, and ultrastructural features in a case. Am J Dermatopathol 1999; 21(4): 395-398.
Kocsard E, Bear CL, Constance TJ. Hyperkeratosis lenticularis perstans (Flegel). Dermatológica 1968; 136(1): 35-42.
Price ML, Jones EW, Macdonald DM. A clinicopathological study of Flegel's disease (hyperkeratosis lenticularis perstans). Br J Dermatol. 1987; 116(5): 681-691.
Urbina F, Sudy E, Misad C. A case of localized, unilateral hyperkeratosis lenticularis perstans on a woman's breast. J Dtsch Dermatol Ges 2016; 14(4): 416-418.
Massone L, Pestarino A, Gambini C, Isola V. Hyperkeratosis lenticularis perstans. A clinical case and review of the literature. G Ital Dermatol Venereol 1990; 125(6): 281-284.
Van de Staak WJ, Bergers AM, Bongaarts P. Hyperkeratosis lenticularis perstans (Flegel). Dermatologica 1980; 161(5): 340-346.
Tidman MJ, Price ML, MacDonald DM. Lamellar bodies in hyperkeratosis lenticularis perstans. J Cutan Pathol 1987; 14(4): 207-211.
Pearson LH, Smith JG, Chalker DK. Hyperkeratosis lenticularis perstans (Flegel's disease). J Am Acad Dermatol 1987; 16(1): 190-195.
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): 255-258.
Errichetti E, Turina M, Pizzolitto S, Stinco G. Dermoscopy of hyperkeratosis lenticularis perstans (Flegel disease). J Dermatol 2019; 46(8): e298-e299.
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; 47(Suppl 1): 38-41.
Fathy S, Azadeh B. Hyperkeratosis lenticularis perstans. Int J Dermatol 1988; 27(2): 120-121.
Bean SF. The genetics of hyperkeratosis lenticularis perstans. Arch Dermatol 1972; 106(1): 72.
Ishibashi A, Tsuboi R, Fujita K. Familial hyperkeratosis lenticularis perstans. Associated with cancers of the digestive organs J Dermatol 1984; 11(4): 407-409.
Ravat F, Ratnavel R. Case 3: widespread scaly papules affecting a patient's limbs and pinnae giving the skin a rough feeling. Diagnosis: Flegel's disease. Syn. hyperkeratosis lenticularis perstans. Clin Exp Dermatol 2003; 28(3): 339-340.
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; 27(5): 812-816.
Ando K, Hattori H, Yamauchi Y. Histopathological differences between early and old lesions of hyperkeratosis lenticularis perstans (Flegel's disease). Am J Dermatopathol 2006; 28(2): 122-126.
Frenk E, Tapernoux B. Hyperkeratosis lenticuiaris perstans (Flegel). A biological model for keratinization occurring in the absence of Odland bodies? Dermatologica 1976; 153(4): 253-262.
Kanitakis J, Hermier C, Hokayem D, Schmitt D. Hyperkeratosis lenticularis perstans (Flegel's disease): a light and electron microscopic study of involved and uninvolved epidermis. Dermatologica 1987; 174(2): 96-101.
Piotrowski MJ, Gunn H, Kirby J, et al. JAAD grand rounds quiz: red-brown hyperkeratotic papules in a 69-year-old man. J Am Acad Dermatol 2015; 73(2): 346-348.
Kuokkanen K, Alavaikko M, Pitkanen R. Hyperkeratosis lenticularis perstans (Flegel's disease). Acta Derm Venereol 1983; 63(4): 357-360.
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; 51(1): 31-35.
Rosdahl I, Rosen K. Hyperkeratosis lenticularis perstans: report on three cases. Acta Derm Venereol 1985; 65(6): 562-564.
Schwarzova K, Kozub P, Szep Z, Golovchenko M, Rudenko N. Detection of Borrelia burgdorferi sensu stricto and Borrelia garinii DNAs in patient with Hyperkeratosis lenticularis perstans (Flegel disease). Folia Microbiol 2016; 61(5): 359-363.
Beveridge GW, Langlands AO. Familial hyperkeratosis lenticularis perstans associated with tumours of the skin. Br J Dermatol 1973; 88(5): 453-458.
Zhang H, Miao C, Zhang X. Hyperkeratosis lenticularis perstans in a patient with primary hyperaldosteronism. J Dtsch Dermatol Ges 2018; 16(1): 72-73.
Valdebran M, Terrero D, Xue R. Dermoscopic findings in hyperkeratosis lenticularis perstans. J Am Acad Dermatol 2016; 75(6): e211-e213.
Russo T, Piccolo V, Mascolo M, Staibano S, Alfano R, Argenziano G. Dermoscopy of Kyrle disease. J Am Acad Dermatol 2016; 75(3): e99-e101.
English JS, Hunter I, Skerrow CJ, Skerrow D, Finlay AY Kyrle-Flegel's - one or two diseases? J R Soc Med 1984; 77(Suppl 4): 16-18.
Donald GF, Hunter GA. Hyperkeratosis Lenticularis Perstans (Flegel), Report of a Case in Which the Application of Betamethasone-17-Valerate Cream Caused Complete Clearing of the Lesions. Australas J Dermatol 1967; 9(1): 31-36.
Wilson PD, Ive F. Treatment of hyperkeratosis lenticularis perstans (Flegel) with topical fluorouracil. Dermatologica 1980; 160(5): 337-340.
Cunha Filho RR, Almeida HL Jr. Hyperkeratosis lenticularis perstans An Bras Dermatol 2011; 86(4 Suppl 1): S76-77.
Bayramgürler D, Apaydin R, Dökmeci S, Ustün M. Flegel's disease: treatment with topical calcipotriol. Clin Exp Dermatol 2002; 27(2): 161-162.
Cooper SM, George S. Flegel's disease treated with psoralen ultraviolet A. Br J Dermatol 2000; 142(2): 340-342.

Auteurs

Paolo Bortoluzzi (P)

Department of Pathophysiology and Transplantation, Università degli Studi di Milano.
Dermatology Unit, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Marco Cusini (M)

Dermatology Unit, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Stefano Veraldi (S)

Department of Pathophysiology and Transplantation, Università degli Studi di Milano.

Gianluca Nazzaro (G)

Dermatology Unit, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

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