The Gorlin-Goltz syndrome: a sporadic case
Kawtar Inani, Fatimazahra Mernissi
The Pan African Medical Journal. 2014;17:55. doi:10.11604/pamj.2014.17.55.3212

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The Gorlin-Goltz syndrome: a sporadic case

Cite this: The Pan African Medical Journal. 2014;17:55. doi:10.11604/pamj.2014.17.55.3212

Received: 11/08/2013 - Accepted: 10/11/2013 - Published: 25/01/2014

Key words: Gorlin-Goltz syndrome, basal cell nevus syndrome, hereditary disorder

© Kawtar Inani et al. The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Corresponding author: Kawtar Inani, Service de Dermatologie, CHU Hassan II, Fez, Morocco (

The Gorlin-Goltz syndrome: a sporadic case


Kawtar Inani1,&, Fatimazahra Mernissi1


1Service de Dermatologie, CHU Hassan II, Fez, Morocco



&Corresponding author
Kawtar Inani, Service de Dermatologie, CHU Hassan II, Fez, Morocco



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Gorlin-Goltz syndrome, also known as basal cell nevus syndrome, is an uncommon autosomal dominant hereditary disorder; which is characterized by numerous basal cell carcinomas, maxillary keratocysts and bones malformations. It results from a mutation of the PATCHED gene. The estimate incidence for a general population is 1/50000 to 1/150000. We present a 30 year-old woman, with no similar familial case, who had a repeatedly dental abscesses since the age of 15 years, she consulted for papulonodular lesions that have appeared at the age of eighty. Those lesions were localized on the face and the trunk, reminiscent of basal cell carcinomas. The clinical examination found papulonodular lesions, a mandibular fistula, broadened nasal root, palmer pits and a swelling at the right big toe. The dermoscopic examination of the papulonodular lesions showed a kind of trunk tree arborisation. The biopsy excision of the swelling at the right toe and papulonodulor lesions, revealed respectively an epidermoid cyst and basal cell carcinomas. Radiographic examination showed a calcified falx cerebri calcification, bifid ribs, synostosis, wedged, scoliosis and agenesis of the left kidney. Our patient was treated by a surgical excision of skin lesion with a setting flat of a mandibular fistula. Also a photoprotection was recommended and a regular monitoring of the skin lesions. The Evolution was marked by the emergence of new basal cell carcinomas treated by photodynamic therapy.

Figure 1: A) papulonodular lesions and a broadened nasal root; B) A mandibular fistula; C)MRI image shows a calcified falx cerebri calcification; D) palmer pits






























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Gorlin-Goltz syndrome
Basal cell nevus syndrome
Hereditary disorder

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