Hyperkeratosis lenticularis perstans (Flegel’s Disease) – Lack of Response to Treatment with Tacalcitol and Calcipotriol

Abstract

Hyperkeratosis lenticularis perstans (HLP) or Flegel’s disease is a rare dermatosis characterized by asymptomatic hyperkeratotic papules predominantly located on the lower extremities. Lesional and non-lesional epidermis samples were studied by light- and electron-microscopic examination. The main ultrastructural finding was the presence of structurally altered Odland bodies/membrane-coating granules. Different therapeutic options for HLP have been reported, but none of the treatments was shown to be consistently effective. Here, we report on a patient with Flegel’s disease who did respond to topical 5-fluorouracil, whereas topical vitamin D₃ synthetics were ineffective.

Histopathological Differences Between Early and Old Lesions of Hyperkeratosis Lenticularis Perstans (Flegel's Disease)

“…4,12 We prescribed topical tacalcitol ointment (Vitamin D 3 synthetics) twice daily for 3 months and the patient showed a slight reduction in the roughness of the lesions.…”

“…2 Removal of the spiny scale above each papule causes slight bleeding. [2][3][4][5][6][7][8][9][10][11][12][13] Although HLP has been considered an autosomal dominant condition, [14][15][16] most cases seem to occur sporadically and usually start in late adult life. 2,4,5,8,[10][11][16][17][18][19] HLP can be clearly distinguished from clinically similar disorders such as Kyrle's disease, porokeratosis and stucco keratosis by its characteristic histologic features.…”

“…2 Previous immunohistochemical features suggested that the composition of the dermal infiltrate contains more CD4-positive T lymphocytes than CD8-positive T lymphocytes. [5][6]12,20 Ultrastructural studies suggested that HLP is a disorder of keratinocyte proliferation with quantitative and/or qualitative alterations in the membrane-coating granules (MCGs) (lamellar granules, Odland bodies) of the keratinocytes, 4,[7][8]10,12,[17][18][19][20][21][22][23] but other authors have reported no abnormalities. 2,13,[24][25] The importance of MCGs in the formation of the epidermal permeability barrier is established.…”

A rare and unusual hyperkeratotic disorder

“…Although the aetiology of the disease is unclear, several studies have proposed different mechanisms of abnormal keratinisation. One hypothesis suggests a defect of the membrane-coating granules (lamellar or Odland bodies)3 as seen on electron microscopy.…”

Hyperkeratosis lenticularis perstans (Flegel's disease) – successful treatment with topical corticosteroids

“…Flegel's disease is primarily a cosmetic affliction. Only in exceptional cases may individuals experience localized pruritus, 1,10 and, on two occasions, a localized unilateral manifestation pattern has been described 11,12 . Disease onset is usually in the fourth or fifth decade of life, and younger individuals are hardly ever affected 5,11 .…”