Monday, April 7, 2014

HISTORY OF XP ..... (1/2)

The road to the current understanding of ‘Xeroderma Pigmentosum’ [XP] started in late 19th century with Dr. Moriz Kaposi, a Hungarian-born professor of dermatology in Vienna. In 1874, Kaposi described 4 patients with ‘Xeroderma’ or ‘‘parchment skin’’ in the early textbook of dermatology (Hebra and Kaposi, 1874), which he wrote with Professor Ferdinand Hebra, his father-in-law (Kraemer et al., 1987). ‘‘In addition to the parchment-like dryness, thinness, and wrinkling of the epidermis, the checkered pigmentation and the small dilatations of the vessels, the most remarkable symptoms were the contraction and, at the same time, thinning of the skin’’, features designating poikiloderma.

In 1883, Albert Neisser of Breslau, Germany reported XP with neurological abnormalities in 2 siblings who had XP with progressive neurological degeneration beginning in the second decade (Neisser, 1883). At present, approximately ~25% of XP patients in the United States develop progressive neurological degeneration (Bradford et al., 2011; Table 1 & Figure 1d).

In 1878, Dr. RW Taylor, MD, of New York reported the first few patients in USA at the inaugural meeting of American Dermatological Association. In 1888, he reviewed the world literature and reported a total of 40 cases (Taylor, 1888).

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