Werner's Syndrome

Reticulohistiocytosis: Resembling Xanthoma Disseminatum

“…Comment: The morphology, distribution and histology suggest either xanthoma disseminatum (regarded as a variety of 'histiocytosis X') or reticulohistiocytosis. Hypercholesterolkmia has rarely been described in xanthoma disseminatum (Koch & Lewis 1956, Smith 1959), but the mild degree found in the present case is not uncommon in reticulohistiocytosis (Warin et al, 1957). However, only one of the 16 cases reviewed by Warin et al developed a rash before bone or joint changes appeared and the distribution of papules in the present case is unusual.…”

Altered Cellular Immunity in Werner’s Syndrome

“…Relevant findings were negative intracutaneous tests to candidin, trichophytin, tuberculin and bacterial antigens, reduced lymphocyte response to PHA, and impaired intracellular killing of Candida albicans by granulocytes. The inclina tion of patients suffering from Werner's syndrome to develop neoplasms may be related to this immunodeficiency.75 years ago, Werner [14] first described a rare, probably autosomal recessive [9,10], slowly progressing disorder, with an onset around the age of 20 [12]. Characteristic features are: small stature, juvenile, in most cases binocular cataracts, scleroderma-like atrophy of the skin of the extremities, particularly involving the distal part of the legs as well as the feet, the sub cutaneous fat and muscles (matchstick legs), relapsing ulcers of the skin areas affected, thin and brittle hair, dystrophic nails, a high-pitched and hoarse voice, hypogonadism, juvenile generalized arteriosclerosis and a 'bird-like' face caused by the loss of subcutaneous fat [3][4][5][6][7], Inconstant features are: diabetes mellitus, warty plantar hyperkeratoses, osteoporosis, splenomegaly, hypothyroidism and disorders of liver metabolism due to liver damage [10].…”