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2023, Number 1

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Invest Medicoquir 2023; 15 (1)

Gorlin-Goltz Syndrome

León GY, Alfonso SC, Prado GK, Bello RI, Lantigua CA, González SY

Language: Spanish
References: 13
Page:
PDF size: 216.56 Kb.


ABSTRACT

Gorlin-Goltz syndrome (GGS) is an autosomal dominant inherited disorder that predisposes mainly to the proliferation of tumors such as basal cell carcinomas and maxillary keratocysts. It is caused by mutation of the Patched gene located on the chromosome. BCCs occurring in patients with GTS are usually multiple, polymorphic in clinical appearance and without sex predilection, generally affecting areas not exposed to sunlight. They show a variable clinical behavior, although sometimes they can be very aggressive, especially at the facial level. We report the case of a patient with Gorlin-Goltz syndrome, a rare and infrequent genetic disorder, difficult to manage in medical practice. Female patient of 61 years of age, operated on more than 120 times for presenting basal cell carcinomas located on the scalp, parpebral edges, anterior and posterior regions of the trunk, with histological subtypes, pigmented, ulcerated and superficial extension. She has innumerable stigmata of Gorlin Goltz Syndrome. For 16 months she has been treated with Heberferon systemically exposing partial and total responses of Basal cell carcinomas. There is insufficient evidence in the literature to determine the treatment of choice for the management of basal cell carcinoma in GCS. Systemic Heberferon is an effective therapeutic alternative in the management of multiple basal cell carcinomas in patients with GBS.


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