Abstract
Malignant atrophic papulosis (MAP) also known as Degos’ disease (Degos et al. 1942; Kohlmeier 1940, 1941) is a rare, often fatal, multisystem disorder characterised by multiple infarcts in the skin and internal organs. The pathognomonic lesions are secondary to narrowing and occlusion of the lumen by intimal proliferation and thrombosis of small-caliber blood vessels which leads to ischaemia and infarction in the involved organ systems (Amato et al. 2005, Pande and Cheskin 2006, Scheinfeld 2006,Torrelo et al. 2002). MAP may involve the gastrointestinal and genitourinary tracts, central and peripheral nervous system, skin, heart, lungs, pancreas, adrenal glands and kidneys. Systemic manifestations usually develop from weeks to years after the onset of skin lesions, or, in rare instances, they may precede the skin lesions (Scheinfeld 2006).
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Schepis, C., Siragusa, M., Amato, C., Ruggieri, M. (2008). Degos’ Disease (Malignant Atrophic Papulosis). In: Ruggieri, M., Pascual-Castroviejo, I., Di Rocco, C. (eds) Neurocutaneous Disorders Phakomatoses and Hamartoneoplastic Syndromes. Springer, Vienna. https://doi.org/10.1007/978-3-211-69500-5_48
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DOI: https://doi.org/10.1007/978-3-211-69500-5_48
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