Kyrle's disease

J Am Acad Dermatol. 1987 Jan;16(1 Pt 1):117-23. doi: 10.1016/s0190-9622(87)70011-5.

Abstract

Kyrle's disease is a chronic, genetically determined, hyperkeratotic disorder occurring in the middle forties. We report fourteen cases of this disorder in a population of 1.5 million over a period of 15 years. We observed a female/male ratio of 6:1. Straw-colored keratotic plugs and flat scale show a marked predisposition for the calf, the tibial region, and the posterior part of the thigh. They vary in size from 1 to 4 mm, and there is occasional plaque formation. No koebnerization or pruritus was noted, and no statistically significant association with hepatic, renal, or diabetic disorders could be demonstrated. Microscopic features showed a consistent pattern of keratotic plug formation, with the plug sitting in an invaginated atrophic epidermis, and underlying dermal histiocyte and lymphocyte aggregation. All lesions contained orthokeratosis and parakeratosis, with some sections showing abnormal keratinization. Sodium dodecylsulfate gel electrophoresis did not demonstrate the presence of abnormal keratin bands.

MeSH terms

  • Adult
  • Aged
  • Darier Disease / epidemiology*
  • Darier Disease / pathology
  • Female
  • Humans
  • Male
  • Middle Aged
  • Northern Ireland
  • Skin / pathology