Un caso di cheratodermia palmo-plantare areata/striata di Siemens

Siemens keratosis palmoplantaris areata/striata, also known as Brunauer- Fuhs keratoderma striatum, is a rare autosomal dominant skin disease classified in the complex and heterogeneous group of hereditary palmar and plantar keratodermas. The underlying defect was recently localised to the linkage of a small gene cluster on chromosome 18q12. The disease is characterized by linear hyperkeratotic lesions on the palms of the hands and islet-shaped lesions on the soles of the feet. The disease is rarely associated with other disorders, such as receptor insensitivity, dysplasia of the dental enamel and modifications of the adnexa. Onset is generally in infancy, when the subject is learning to walk, suggesting that the lesions are triggered by mechanical stimulation. A case of Siemens keratosis palmoplantaris areata/striata in a 46 year-old woman is described. The subject had linear hyperkeratotic lesions on the palms and islet-shaped lesions on the soles since the age of two years. Family medical history revealed that the father also had similar lesions. Histological examination of lesional tissue showed hyperkeratosis with focal parakeratosis, acanthosis, papillomatosis and hypergranulosis. The diagnosis and classification are discussed with special reference to the Siemens variant.