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Unilateral Asymptomatic Progressive Eruption of Hyperkeratotic Papules

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A man in his 60s presented with a 10-year history of a slowly progressive, asymptomatic cutaneous eruption on his left leg (Figure 1A). He had a 16-year history of diabetes mellitus that was being treated with metformin. Hematological and biochemical test results were unremarkable. There was no history of another endocrine disorder or malignant neoplasm. There was no family history of similar cutaneous findings. Physical examination revealed reddish brown hyperkeratotic papules 1 to 10 mm wide on the front and back of the left leg from knee to ankle. The rest of the physical examination findings were unremarkable. Removal of the scales caused slight bleeding. A lesional skin biopsy was performed (Figure 1B and C).

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B. Hyperkeratosis lenticularis perstans

Histopathologic examination revealed focal epidermal atrophy, orthokeratotic hyperkeratosis, and a bandlike infiltrate of lymphocytes in the superficial dermis compatible with unilateral hyperkeratosis lenticularis perstans (HLP) (Figure 1B and C). He was treated with acitretin and topical tacalcitol with substantial improvement of his cutaneous lesions (Figure 2).

Described by Flegel1 in 1958, HLP is a disease of uncertain origin, though it has been shown to have an autosomal dominant inheritance pattern with late onset.2 This rare disorder is characterized by numerous symmetric hyperkeratotic papules. Lesions are most common on the distal portion of the legs and dorsal surface of the feet. Arms, palms, and soles can be affected. Other parts of the body, including the oral mucosa, are rarely affected, and the trunk is usually spared.3 The patient has localized unilateral HLP, which is a rarely seen form of the disease4,5 The lesions are usually hyperkeratotic papules 1 to 5 mm in diameter, with an initial erythematous coloration that with time acquires brownish coloration. When the papules are dislodged, they give rise to a depression with punctate bleeding. It runs a chronic course, and lesions persist indefinitely.

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Article Information

Corresponding Author: Javier García-Abellán, MD, Department of Internal Medicine, Hospital General Universitario de Elche, Plaza Yesqueros number 3, 1B, Murcia (Spain), Elche 30005, Spain (javiergarciaabellan@hotmail.com).

Published Online: April 24, 2019. doi:10.1001/jamadermatol.2019.0308

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Flegel  H.  Hyperkeratosis lenticularis perstans.  Hautarzt. 1958;9(8):363-364.PubMedGoogle Scholar
2.
Bean  SF.  Hyperkeratosis lenticularis perstans: a clinical, histopathologic, and genetic study.  Arch Dermatol. 1969;99(6):705-709. doi:10.1001/archderm.1969.01610240063011PubMedGoogle ScholarCrossref
3.
Pearson  LH, Smith  JG  Jr, Chalker  DK.  Hyperkeratosis lenticularis perstans (Flegel’s disease): case report and literature review.  J Am Acad Dermatol. 1987;16(1 Pt 2):190-195. doi:10.1016/S0190-9622(87)80060-9PubMedGoogle ScholarCrossref
4.
Miranda-Romero  A, Sánchez Sambucety  P, Bajo del Pozo  C, Martinez Fermandez  M, Esquivias Gómez  JI, Garcia Muñoz  M.  Unilateral hyperkeratosis lenticularis perstans (Flegel’s disease).  J Am Acad Dermatol. 1998;39(4 Pt 1):655-657. doi:10.1016/S0190-9622(98)70023-4PubMedGoogle ScholarCrossref
5.
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.PubMedGoogle Scholar
6.
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. doi:10.1097/01.dad.0000181544.50959.aePubMedGoogle ScholarCrossref
7.
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 Pt 2):812-816. doi:10.1016/0190-9622(92)70254-DPubMedGoogle ScholarCrossref
8.
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. doi:10.1159/000051648PubMedGoogle ScholarCrossref
9.
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;6(3):93-95. doi:10.3315/jdcr.2012.1112PubMedGoogle ScholarCrossref
10.
Gabrielsen  TO.  Tigason in hyperkeratosis lenticularis perstans (HLP)—a case report [in German].  Z Hautkr. 1986;61(4):214-217.PubMedGoogle Scholar
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