Kyrle-flegel disease in siblings with childhood onset and koebner phenomenon

Abstract

This is a report of Kyrle-Flegel disease occurring in 3 siblings. Two of them were girls (13 and 9 years) and the third patient was a boy (7 years). They had developed hyperkeratotic papules since the ages of 9, 6 and 5 years respectively. Lesions were present mainly on the extensor surfaces of limbs. Some lesions were linear, they had appeared at the sites of trauma. Histology showed laminated hyperkeratosis, follicular plugging and flattening of the dermoepidermal junction. Patients partially responded to oral vitamin A in high doses.