Phacomatosis Pigmentokeratotica: A 20‐Year Follow‐up with Malignant Degeneration of Both Nevus Components
: Phacomatosis pigmentokeratotica is a rare syndrome defined by the association of an organoid nevus occasionally with sebaceous differentiation, a speckled lentiginous nevus, and other extracutaneous anomalies. The disorder is a consequence of the so‐called twin spot genetic mechanism. We describe...
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Veröffentlicht in: | Pediatric dermatology 2005-01, Vol.22 (1), p.44-47 |
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creator | Martínez‐Menchón, Teresa Mahiques Santos, L. Vilata Corell, J. J. Febrer Bosch, I. Fortea Baixauli, J. M. |
description | : Phacomatosis pigmentokeratotica is a rare syndrome defined by the association of an organoid nevus occasionally with sebaceous differentiation, a speckled lentiginous nevus, and other extracutaneous anomalies. The disorder is a consequence of the so‐called twin spot genetic mechanism. We describe the first occurrence involving malignant degeneration of both nevus components, giving rise to three basal cell carcinomas over the sebaceous nevus and a malignant melanoma of the superficial spreading type over the speckled lentiginous nevus. This observation, in concert with the other instances reported in the literature, points to the need for adequate patient follow‐up to ensure early detection and treatment of any possible associated malignant degeneration. |
doi_str_mv | 10.1111/j.1525-1470.2005.22110.x |
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J.</creatorcontrib><creatorcontrib>Febrer Bosch, I.</creatorcontrib><creatorcontrib>Fortea Baixauli, J. M.</creatorcontrib><title>Phacomatosis Pigmentokeratotica: A 20‐Year Follow‐up with Malignant Degeneration of Both Nevus Components</title><title>Pediatric dermatology</title><addtitle>Pediatr Dermatol</addtitle><description>: Phacomatosis pigmentokeratotica is a rare syndrome defined by the association of an organoid nevus occasionally with sebaceous differentiation, a speckled lentiginous nevus, and other extracutaneous anomalies. The disorder is a consequence of the so‐called twin spot genetic mechanism. We describe the first occurrence involving malignant degeneration of both nevus components, giving rise to three basal cell carcinomas over the sebaceous nevus and a malignant melanoma of the superficial spreading type over the speckled lentiginous nevus. This observation, in concert with the other instances reported in the literature, points to the need for adequate patient follow‐up to ensure early detection and treatment of any possible associated malignant degeneration.</description><subject>Adult</subject><subject>Biological and medical sciences</subject><subject>Carcinoma, Basal Cell - complications</subject><subject>Carcinoma, Basal Cell - physiopathology</subject><subject>Cell Transformation, Neoplastic</subject><subject>Dermatology</subject><subject>Disease Progression</subject><subject>Follow-Up Studies</subject><subject>Humans</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Melanoma - complications</subject><subject>Melanoma - physiopathology</subject><subject>Neurocutaneous Syndromes - complications</subject><subject>Neurocutaneous Syndromes - physiopathology</subject><subject>Nevus, Pigmented - complications</subject><subject>Nevus, Pigmented - physiopathology</subject><subject>Pigmentary diseases of the skin</subject><subject>Skin Neoplasms - complications</subject><subject>Skin Neoplasms - physiopathology</subject><subject>Tumors of the skin and soft tissue. 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M.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3980-88afaf0f1fade9994e263617eb0cb8e9fbd9be170d3110a58c2451f4060a356a3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2005</creationdate><topic>Adult</topic><topic>Biological and medical sciences</topic><topic>Carcinoma, Basal Cell - complications</topic><topic>Carcinoma, Basal Cell - physiopathology</topic><topic>Cell Transformation, Neoplastic</topic><topic>Dermatology</topic><topic>Disease Progression</topic><topic>Follow-Up Studies</topic><topic>Humans</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Melanoma - complications</topic><topic>Melanoma - physiopathology</topic><topic>Neurocutaneous Syndromes - complications</topic><topic>Neurocutaneous Syndromes - physiopathology</topic><topic>Nevus, Pigmented - complications</topic><topic>Nevus, Pigmented - physiopathology</topic><topic>Pigmentary diseases of the skin</topic><topic>Skin Neoplasms - complications</topic><topic>Skin Neoplasms - physiopathology</topic><topic>Tumors of the skin and soft tissue. Premalignant lesions</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Martínez‐Menchón, Teresa</creatorcontrib><creatorcontrib>Mahiques Santos, L.</creatorcontrib><creatorcontrib>Vilata Corell, J. J.</creatorcontrib><creatorcontrib>Febrer Bosch, I.</creatorcontrib><creatorcontrib>Fortea Baixauli, J. M.</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatric dermatology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Martínez‐Menchón, Teresa</au><au>Mahiques Santos, L.</au><au>Vilata Corell, J. J.</au><au>Febrer Bosch, I.</au><au>Fortea Baixauli, J. M.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Phacomatosis Pigmentokeratotica: A 20‐Year Follow‐up with Malignant Degeneration of Both Nevus Components</atitle><jtitle>Pediatric dermatology</jtitle><addtitle>Pediatr Dermatol</addtitle><date>2005-01</date><risdate>2005</risdate><volume>22</volume><issue>1</issue><spage>44</spage><epage>47</epage><pages>44-47</pages><issn>0736-8046</issn><eissn>1525-1470</eissn><coden>PEDRDQ</coden><abstract>: Phacomatosis pigmentokeratotica is a rare syndrome defined by the association of an organoid nevus occasionally with sebaceous differentiation, a speckled lentiginous nevus, and other extracutaneous anomalies. The disorder is a consequence of the so‐called twin spot genetic mechanism. We describe the first occurrence involving malignant degeneration of both nevus components, giving rise to three basal cell carcinomas over the sebaceous nevus and a malignant melanoma of the superficial spreading type over the speckled lentiginous nevus. This observation, in concert with the other instances reported in the literature, points to the need for adequate patient follow‐up to ensure early detection and treatment of any possible associated malignant degeneration.</abstract><cop>Oxford, UK</cop><pub>Blackwell Science Inc</pub><pmid>15660897</pmid><doi>10.1111/j.1525-1470.2005.22110.x</doi><tpages>4</tpages></addata></record> |
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subjects | Adult Biological and medical sciences Carcinoma, Basal Cell - complications Carcinoma, Basal Cell - physiopathology Cell Transformation, Neoplastic Dermatology Disease Progression Follow-Up Studies Humans Male Medical sciences Melanoma - complications Melanoma - physiopathology Neurocutaneous Syndromes - complications Neurocutaneous Syndromes - physiopathology Nevus, Pigmented - complications Nevus, Pigmented - physiopathology Pigmentary diseases of the skin Skin Neoplasms - complications Skin Neoplasms - physiopathology Tumors of the skin and soft tissue. Premalignant lesions |
title | Phacomatosis Pigmentokeratotica: A 20‐Year Follow‐up with Malignant Degeneration of Both Nevus Components |
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