Gorlin-Goltz syndrome in an older black adult. Case Report

Introduction: Gorlin-Goltz syndrome (GGS), or basal cell nevus syndrome (BCNS), is a rare genetic disease that induces the development of odontogenic keratocysts, skeletal malformations and neoplasms, especially multiple and recurrent basal cell carcinomas (BCC). This condition is rare in black peo...

Full description

Saved in:
Bibliographic Details
Main Authors: Mauricio Andrés Uribe-Valencia, Janeth Jurado-Delgado, Lina Marcela Velásquez-Tafur, Luis Fernando Cárdenas-Cardona
Format: Article
Language:English
Published: Universidad Nacional de Colombia 2021-04-01
Series:Revista de la Facultad de Medicina
Subjects:
Online Access:https://revistas.unal.edu.co/index.php/revfacmed/article/view/83066
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1849699025086840832
author Mauricio Andrés Uribe-Valencia
Janeth Jurado-Delgado
Lina Marcela Velásquez-Tafur
Luis Fernando Cárdenas-Cardona
author_facet Mauricio Andrés Uribe-Valencia
Janeth Jurado-Delgado
Lina Marcela Velásquez-Tafur
Luis Fernando Cárdenas-Cardona
author_sort Mauricio Andrés Uribe-Valencia
collection DOAJ
description Introduction: Gorlin-Goltz syndrome (GGS), or basal cell nevus syndrome (BCNS), is a rare genetic disease that induces the development of odontogenic keratocysts, skeletal malformations and neoplasms, especially multiple and recurrent basal cell carcinomas (BCC). This condition is rare in black people, being reported in this population in only 5% of the cases. Case presentation: A 68-year-old black man reported the constant appearance for approximately 4 years of multiple papules and non-pruritic and non-desquamating skin plaques, with hyperpigmented margins, of different sizes that grew gradually in scalp, left lower eyelid, arms, forearms, back, and lower limbs. Histopathological study showed multiple BCC, and imaging studies identified calcifications in the tentorium cerebelli and cerebral falx, as well as images suggestive of odontogenic cysts. Based on his clinical history, histopathologic and imaging findings, and physical examination, he was diagnosed with GGS. Conclusions: This is the first case of GGS in an older black adult reported in Colombia. This case highlights the relevance of reviewing the medical records and performing a thorough physical examination when approaching the patient, as well as doing a comprehensive geriatric assessment, since they are key to diagnose this rare disease and initiate a timely multidisciplinary treatment. This will allow obtaining better outcomes in these patients.
format Article
id doaj-art-0bbdccb958b14fa9a61d2e296c85163d
institution DOAJ
issn 0120-0011
2357-3848
language English
publishDate 2021-04-01
publisher Universidad Nacional de Colombia
record_format Article
series Revista de la Facultad de Medicina
spelling doaj-art-0bbdccb958b14fa9a61d2e296c85163d2025-08-20T03:18:43ZengUniversidad Nacional de ColombiaRevista de la Facultad de Medicina0120-00112357-38482021-04-0169210.15446/revfacmed.v69n2.83066Gorlin-Goltz syndrome in an older black adult. Case ReportMauricio Andrés Uribe-Valencia0https://orcid.org/0000-0002-0065-6152Janeth Jurado-Delgado1https://orcid.org/0000-0002-0305-1714Lina Marcela Velásquez-Tafur2https://orcid.org/0000-0003-4418-7092Luis Fernando Cárdenas-Cardona3https://orcid.org/0000-0002-7244-6234Universidad del Valle - Faculty of Health - Geriatrics Program - Cali - Colombia.Universidad del Valle - Faculty of Health - Geriatrics Program - Cali - Colombia.Universidad del Valle - Faculty of Health - Department of Internal Medicine - Dermatology and Dermatological Surgery Section - Cali - Colombia.Universidad del Valle - Faculty of Health - Department of Internal Medicine - Dermatology and Dermatological Surgery Section - Cali - Colombia. Introduction: Gorlin-Goltz syndrome (GGS), or basal cell nevus syndrome (BCNS), is a rare genetic disease that induces the development of odontogenic keratocysts, skeletal malformations and neoplasms, especially multiple and recurrent basal cell carcinomas (BCC). This condition is rare in black people, being reported in this population in only 5% of the cases. Case presentation: A 68-year-old black man reported the constant appearance for approximately 4 years of multiple papules and non-pruritic and non-desquamating skin plaques, with hyperpigmented margins, of different sizes that grew gradually in scalp, left lower eyelid, arms, forearms, back, and lower limbs. Histopathological study showed multiple BCC, and imaging studies identified calcifications in the tentorium cerebelli and cerebral falx, as well as images suggestive of odontogenic cysts. Based on his clinical history, histopathologic and imaging findings, and physical examination, he was diagnosed with GGS. Conclusions: This is the first case of GGS in an older black adult reported in Colombia. This case highlights the relevance of reviewing the medical records and performing a thorough physical examination when approaching the patient, as well as doing a comprehensive geriatric assessment, since they are key to diagnose this rare disease and initiate a timely multidisciplinary treatment. This will allow obtaining better outcomes in these patients. https://revistas.unal.edu.co/index.php/revfacmed/article/view/83066Basal Cell CarcinomaGorlin-Goltz SyndromeBasal cell Nevus SyndromeElderly
spellingShingle Mauricio Andrés Uribe-Valencia
Janeth Jurado-Delgado
Lina Marcela Velásquez-Tafur
Luis Fernando Cárdenas-Cardona
Gorlin-Goltz syndrome in an older black adult. Case Report
Revista de la Facultad de Medicina
Basal Cell Carcinoma
Gorlin-Goltz Syndrome
Basal cell Nevus Syndrome
Elderly
title Gorlin-Goltz syndrome in an older black adult. Case Report
title_full Gorlin-Goltz syndrome in an older black adult. Case Report
title_fullStr Gorlin-Goltz syndrome in an older black adult. Case Report
title_full_unstemmed Gorlin-Goltz syndrome in an older black adult. Case Report
title_short Gorlin-Goltz syndrome in an older black adult. Case Report
title_sort gorlin goltz syndrome in an older black adult case report
topic Basal Cell Carcinoma
Gorlin-Goltz Syndrome
Basal cell Nevus Syndrome
Elderly
url https://revistas.unal.edu.co/index.php/revfacmed/article/view/83066
work_keys_str_mv AT mauricioandresuribevalencia gorlingoltzsyndromeinanolderblackadultcasereport
AT janethjuradodelgado gorlingoltzsyndromeinanolderblackadultcasereport
AT linamarcelavelasqueztafur gorlingoltzsyndromeinanolderblackadultcasereport
AT luisfernandocardenascardona gorlingoltzsyndromeinanolderblackadultcasereport