Kyrle's disease

Abstract

Hyperkeratosis follicularis et parafollicularis in cuten penetrans known as Kyrle's disease is a recessive hereditary genodermatosis. Generally, Kyrle's disease appears between 30 to 50 years of age and women are affected more than men. We report a case of a 40-year-old man, a worker in a cement factory, who noticed the first skin lesions six years ago. Those were follicular papules on the left leg. He had been in good health beforehand, although an alcoholic for many years. Based on laboratory and other findings, chronic hepatitis was diagnosed. Histopathological analysis of skin lesions showed presence of hyperkeratosis and partial parakeratosis with acanthosis of the epidermis. Kyrle's disease is classified among the perforating dermatoses with perforating folliculitis, perforating serpiginous elastosis and perforating reactive collagenosis. Kyrle's disease is often associated with hepatic, renal or diabetic disorders, and may be a paraneoplastic disease. The course of this disease is chronic. If the associated diseases are treated, patients may have a better prognosis.