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Image Number 1000 (Gorlin's syndrome)

 
Morphology:
Nodule

Diagnosis: Gorlin's syndrome

Site: Face

Sex: M

Age: 57

Type: Clinical

Description: Mutiple small noduloulcerative BCCs, some had left scars after treatment.

Submitted by: Nameer Al-Sudany

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Differential Diagnosis

History:

Gorlin’s syndrome (Naevoid basal cell carcinoma syndrome) is characterized by multiple basal cell carcinomas and may be associated with palmoplantar pits and also, epidermal cysts are quite common. Most patients show multiple skeletal and central nervous system anomalies among which are odontogenic keratocysts of the jaws, anomalies of the ribs, scoliosis, mental retardation, and calcification of the falx cerebri. The syndrome is inherited as an autosomal dominant trait with low penetration.

This 57-year-old man suffered the appearance of numerous small nodules at age of 15 years mainly on the face. The nodules slowly increased in number and size. Few years later many of these nodules became ulcerative. Also, numerous palmoplantar pits few millimeters in diameter had appeared at age of twenties. A jaw cyst had been removed by a maxillofacial surgeon 10 years ago. One of the brothers of the patient also had a similar condition.



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