What are the other Names for this Condition? (Also known as/Synonyms)

• Natal Teeth and Steatocystoma Multiplex

What is Steatocystoma Multiplex with Natal Teeth? (Definition/Background Information)

• Steatocystoma Multiplex with Natal Teeth is an uncommon genetic disorder that is characterized by numerous steatocystoma cysts on the body, along-with underdeveloped teeth at birth (natal teeth). The disorder has been only described in a Chinese family that spanned several generations
• In many individuals, the presence of numerous lesions widespread in the body may cause constant cosmetic concerns. The treatment of Steatocystoma Multiplex with Natal Teeth may involve the administration of oral medications, cryotherapy, and removal of cysts via surgery; natal teeth may be extracted as recommended by a dental expert. The overall prognosis is generally good, but this may vary from one individual to another

Who gets Steatocystoma Multiplex with Natal Teeth? (Age and Sex Distribution)

• Steatocystoma Multiplex with Natal Teeth is an extremely rare congenital disorder, described so far in only 21 members of a single family (spanning 5 generations)
• The onset of symptoms may occur at or following the birth of the child
• Both genders may be affected
• No data is available regarding the occurrence of this condition worldwide. Almost all reported cases are in individuals of Chinese descent

What are the Risk Factors for Steatocystoma Multiplex with Natal Teeth? (Predisposing Factors)

• A positive family history may be an important risk factor, since Steatocystoma Multiplex with Natal Teeth can be inherited
• Individuals of certain Chinese descent are known to have a high risk for this disorder

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Steatocystoma Multiplex with Natal Teeth? (Etiology)

• Presently, the genetic cause of Steatocystoma Multiplex with Natal Teeth is not well-established
• The condition is reportedly inherited in an autosomal dominant manner

Autosomal dominant inheritance: Autosomal dominant conditions are traits or disorders that are present when only one copy of the mutation is inherited on a non-sex chromosome. In these types of conditions, the individual has one normal copy and one mutant copy of the gene. The abnormal gene dominates, masking the effects of the correctly function gene. If an individual has an autosomal dominant condition, the chance of passing the abnormal gene on to their offspring is 50%. Children, who do not inherit the abnormal gene, will not develop the condition or pass it on to their offspring.

What are the Signs and Symptoms of Steatocystoma Multiplex with Natal Teeth?

The signs and symptoms of Steatocystoma Multiplex with Natal Teeth may vary among affected individuals in type and severity.

• Presence of multiple cysts on the skin, involving the chest, back, limbs, and face, among other sites
• The cysts may be filled with oily fluid (white-yellow in color)
• Infected cysts can be painful
• Nail abnormalities
• Natal tooth - presence of underdeveloped teeth at birth

How is Steatocystoma Multiplex with Natal Teeth Diagnosed?

Steatocystoma Multiplex with Natal Teeth is diagnosed on the basis of the following information:

• A complete evaluation of family and personal medical history along with a thorough examination of the skin lesions by a dermatologist
• Blood tests and culture studies, if necessary
• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Skin biopsy: A skin tissue biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Steatocystoma Multiplex with Natal Teeth?

The complications of Steatocystoma Multiplex with Natal Teeth may include:

• Emotional stress
• Feeding issues
• Secondary bacterial and fungal infections
• Cosmetic issues from scarring on healing of the lesions
• Low self-esteem

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Steatocystoma Multiplex with Natal Teeth Treated?

There is no cure for ASteatocystoma Multiplex with Natal Teeth, since it is a genetic condition. There is also no well-defined treatments available for the condition. In many, the treatment is usually given to manage the signs and symptoms and any complication that develops.

The healthcare provider may consider any of the following measures:

• Oral isotretinoin
• Oral antibiotic therapy
• Cyst aspiration and drainage
• Liquid nitrogen cryotherapy
• Dermabrasion
• Carbon dioxide laser therapy - especially when numerous cysts are seen
• Surgical excision and cyst removal
• Evaluation of natal teeth by a dentist with teeth extraction, as needed

Regular medical screening at periodic intervals with tests and physical examinations are recommended.

How can Steatocystoma Multiplex with Natal Teeth be Prevented?

Steatocystoma Multiplex with Natal Teeth may not be preventable, since it is a genetic disorder.

• Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
• If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders

What is the Prognosis of Steatocystoma Multiplex with Natal Teeth? (Outcomes/Resolutions)

• The prognosis of Steatocystoma Multiplex with Natal Teeth is dependent upon the severity of the signs and symptoms and associated complications, if any
• In most cases, the condition is typically asymptomatic, although the presence of a large number of cysts can cause chronic cosmetic issues

Additional and Relevant Useful Information for Steatocystoma Multiplex with Natal Teeth:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/healthy-living/skin-disorders/