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Gorlin Syndrome Group

Supporting patients, their families and carers
 
Registered Charity No: 1096361
Supporting patients, their families, friends and carers affected by Gorlin Syndrome
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Skin Problems in Gorlin Syndrome
Listed below are the various problems experienced by many people affected by Gorlin Syndrome.



Basal cell naevi and basal cell carcinoma (BCC)

If a 'naevus' and a basal cell carcinoma from someone with Gorlin Syndrome are examined under a microscope, their appearances are identical. However, they behave differently.

The 'naevi' often develop first, sometimes BCCs appear to arise from naevi. For the purposes of the description which follows, it is helpful to treat 'naevi' and 'naevoid basal cell carcinomas' as thought they were separate entities.


Naevi

Naevi affect 53% of patients less than 20 years of age, rising to 74% over the age of 20 years. Ordinary naevus cell naevi (moles) occur in about 4% of unaffected relatives and are present from birth, whilst affected family members report that the naevi tend to occur multiply in crops, their numbers increasing with time. Naevi also appear as individual lesions. Someone with Gorlin Syndrome may develop no naevi, a few or many hundreds.

The naevi are flesh coloured, reddish brown or pearly, the groups resembling moles, skin tags, ordianry naevus cell naevi or haemangoimas. Some grow rapidly for a few days to a few weeks, but the most remain static. If one is removed, it will have the appearance down the microscope of a basal cell carcinoma, which can cause great alarm in children with the syndrome. In fact only 14% of people with Gorlin Syndrome under the age of 20 years have a rapidly growing BCC. It is unusual to develop aggressive BCCs before puberty.


Skin tags

Skin tags are especially common around the neck, like the naevi, histology reveals the typical features of a basal cell carcinoma, but the skin tags do not generally change in size or shape.


Cysts in the skin

Small keratin filled cysts (milia) are found on the face in 30%, most commonly in the area below the eyes, but they can also occur on the forehead.

Meibornian cysts on the corneal surface of the eyelids can be distressing as they repeatedly discharge material.

Epidermoid cysts occur on the limbs and trunk in over 50% of cases. They are usually 1-2 cms in diameter, and are particularly common around the knee.



Important Information

Basal Cell Carcinomas
 
BCCs are discussed at further length on the page entitled
Basal Cell Carcinomas
 
Remember BCCs are curable if treated early. 
 

Treatment Options
 
Many treatments exist for BCCs and will depend upon the age of the patient and the size, location and extent of the lesion.
 
To ensure the effective management of Gorlin Syndrome it is vital that patients are treated in the early stages. 
 
A range of options are listed on the Treatment Options page, which can be accessed on the menu to left of this page.
 
 




Regularly inspect your skin
Annual Surveillance
 
Of the skin by a Plastic Surgeon or Dermatologist is desirable in order that timely treatment is provided.
 
It is also recommended that patients with Gorlin Syndrome are seen on a regular basis by an experienced Dermatologist. 
 
 

Discuss treatment options
 
With the specialist looking after your care to ascertain which treatment would best suit your individual situation.
 
If you have worries or concerns speak to the specialist taking care of your treatment.  
 
Contact Us if you require any further information. 
 
 

 




 
 
 
Written and produced by Professor P A Farndon, Clinical Geneticist at the National Genetics Education and Development Centre UK, Jim Costello (deceased) & Margaret Costello, unless otherwise stated.