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
Hauptverfasser: Martínez‐Menchón, Teresa, Mahiques Santos, L., Vilata Corell, J. J., Febrer Bosch, I., Fortea Baixauli, J. M.
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container_end_page 47
container_issue 1
container_start_page 44
container_title Pediatric dermatology
container_volume 22
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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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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