Keratoderma hereditaria mutilans. Etretinate treatment and electron microscope studies.

摘要: Summary Keratoderma hereditaria mutilans is a rare inherited cornification disorder characterized by hyperkeratosis of palms and soles with characteristic “honey-comb” appearance, keratotic constriction furrows one or more digits (pseudo-ainhum) threatening spontaneous amputation. Approximately 30 cases have been reported, but the ultrastructural features not well characterized. In this article, we describe clinical, histologic, findings in our patient. A biopsy specimen taken before treatment etretinate showed well-preserved granular layer acanthosis. Ultrastructural examination disclosed that spinous cells contained marked swollon mitochondria, many desmosomes, corneocytes membrane coating granules (MCG) lipid like vacuoles. After initiation treatment, considerable clinical improvement was observed, without significant histologic modification. The principal post-treatment changes were reduction mitochondrial swelling number MCG incorneocytes. intercellular spaces increased amounts fine coarse substances. Stimulation Langerhans observed. Marginal band formation occurred normally after treatment.