RESUMEN
Xanthoma disseminatum is a rare, benign, normolipemic form of non-Langerhans cell histiocytosis affecting the skin and mucous membranes. The cutaneous manifestations consist of hundreds of papules that are red – brown at first and then become yellowish. The papules symmetrically involve the eyelids, trunk, face and proximal extremities and in flexures and folds, tend quickly to merge, forming soft plaques. It is frequently associated with diabetes insipidus. Biopsy specimens show a mixture of histiocytes, foam cells, and inflammatory cells. Later, foam cells predominate and Touton giant cells are frequently present. This chronic disease has no known established treatment.
RESUMEN
Papillon–Lefevre syndrome is an extremely rare autosomal recessive disorder characterized by severe periodontal disease with hyperkeratosis and fissuring of the palms and soles. Periodontitis is severe and destructive affecting both deciduous and permanent dentitions associated with palmo-plantar hyperkeratosis. These manifestations usually appear in childhood between 1 and 4 years of age and deciduous teeth exfoliate within or at the age of six. Permanent teeth erupt normally but soon get affected by periodontal disease. Individual becomes edentulous within teenage. Hyperkeratotic lesions extend to knees and elbows. Some cases have shown inconsistent manifestations such as calcification of falx cerebri and choroid plexus, calcification of the dura, attachment of the tentorium, thumb nail dystrophy and hyperhidrosis.