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Titlebook: Deadly Dermatologic Diseases; Clinicopathologic At David R. Crowe,Michael Morgan,Kara Trapp Book 2016Latest edition Springer International

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Andrea Bandini,Patrizia Gianni,Enrico Sbarraentail the development of multiple cutaneous and mucous membrane vascular lesions associated with other life-threatening complications. This chapter deals with the clinical and pathologic features of three such conditions, namely, Osler-Weber-Rendu (OWR) syndrome, ataxia-telangiectasia (AT), and Fabry’s disease (FD).
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Paget’s Diseasesement membrane of the underlying ducts to the epidermis of the nipple. The second theory, in situ malignant transformation or degeneration from existing cells, postulates that the Paget cell is a malignant keratinocyte in situ; thus, this theory defines Paget’s disease as an in situ carcinoma without underlying cancer of the breast.
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Gardner Syndromes. Interestingly, the cutaneous lesions were not characterized in that report because one family member had expired shortly after having a cutaneous lesion removed, and others in the family were afraid they would meet the same fate if their lesions were removed.
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Mast Cell Disease: Urticaria Pigmentosane c-kit at codon 816. C-kit functions as a transmembrane protein and is involved in signaling cell division when bound to mast cell growth factor. Mutations in the 816 position result in abnormal proliferation of mast cells.
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