Steatocystoma Multiplex
Steatocystoma multiplex was first described in 1873 by Sir William Allan Jamieson, a Scottish physician who presented a case of numerous skin cyst scattered all over the body. Steatocystoma multiplex (SM) remains rare, but for individuals with this unusual skin condition, it causes significant distress. It is perhaps the true sebaceous cysts (unlike the common epidermal cysts), as this benign condition is thought to arise from the lining of the ducts of the sebaceous gland.
what is steatocystoma multiplex?
Steatocystoma multiplex is a rare genetic disorder characterized by the development of multiple sebaceous cysts, typically arising during adolescence or early adulthood. These cysts, which are benign, usually manifest on the trunk, upper arms, and sometimes on the neck or face. The condition is inherited in an autosomal dominant pattern, meaning a single copy of the altered gene in each cell is sufficient to cause the disorder. The cysts are often asymptomatic, but they can become inflamed, infected, or painful, necessitating medical intervention. Treatment options focus on managing symptoms and may include surgical removal, laser therapy, or oral medications to reduce inflammation.
What causes Steatocystoma Multiplex?
Most cases of steatocystoma multiplex begins during puberty. There is probably a hormonal trigger in susceptible individuals with this condition. In most cases, there is no history of steatocystoma multiplex that runs in the family, it happens sporadically. When there is a positive family history of steatocystoma multiplex in the same family, some studies have identified that a genetic mutation of the Keratin 17 (KRT17) gene may be responsible. This gene is normally found in the sebaceous gland, nail bed and hair follicles.
Diagnosis
- Clinical Examination: Diagnosis is primarily based on clinical evaluation and the characteristic appearance of the cysts.
- Histopathology: A biopsy and microscopic examination of the cysts can confirm the diagnosis. The cysts typically show a lining of stratified squamous epithelium with a well-formed granular layer, and they contain sebaceous material.
- Genetic Testing: Genetic testing can identify mutations in the KRT17 gene, providing a definitive diagnosis.
What does it look like?
Although steatocystoma multiplex can occur all over the body, there is a preference for body sites where the sebaceous glands are highly concentrated. They are often found on the neck, chest, armpits and arms. The cysts range from 3mm to 3cm in diameter, are mobile within the skin and have varying depths. Superficial cysts tend to take on a yellowish appearance. Unlike epidermal cysts, in which an opening (punctum) of the skin is found, this is absent in steatocystoma multiplex.
More often than not, the cysts do not give rise to symptoms such as pain or itchiness. However, there can be occasions where the cysts gets inflammed or infected. When this happens, the skin cyst may be swollen, tender, red or even discharge pus. It is advisable to see a medical professional at this stage for treatment.
Steatocystoma Multiplex Treatments
1. Medications
There have been attempts to use medications to treat SM, but the results have been far from encouraging. It would seem logical to use oral isotretinoin to treat this condition, as this drug, which is extremely effective at clearing acne reduces the size of the sebaceous gland. However, the reports of the use of oral isotretinoin have not been consistent, with some individuals even reporting flares and recurrences following medical therapy.
2. Aspiration
Aspiration is a technique of removing the cystic contents under negative pressure using a sterile needle. However, the cystic wall remains within the skin, and it only a matter of time before the cysts becomes apparent.
3. Incision & Drainage
This is a technique where a blade is used to create an entry point into the skin, and the cysts contents are then squeezed out under pressure. This procedure is not ideal for the treatment of SM. It is associated with a high recurrence rate as the cysts wall remains intact within the skin. The incision & drainage procedure is best reserved for cases where the cysts has become infected and pus has accumulated, requiring surgical drainage.
4. Modified Incisional Technique
In this modified technique, we are able to remove the cyst wall completely with no recurrence after the procedure. Under local anaesthesia, a small incision 2-3mm is placed over the skin, the cystic walls are gently dissected under magnification. There are various ways to remove the cystic wall, and the use of a vein hook or edge of a fine tooth forceps is one my personal favourites. Scarring is almost imperceptible as the incision is small, and often hidden within the natural skin lines.
5. Laser Surgery
Carbon dioxide laser surgery is another option to treat SM. However, it is often difficult to remove the cystic wall and contents completely with this procedure alone, and recurrence remains a problem.
Outcomes
- Chronic Condition: Steatocystoma multiplex is a chronic condition that can persist throughout life. While benign, the cysts can cause significant cosmetic and psychological impact.
- Regular Monitoring: Regular follow-up is advisable to manage any complications and monitor for new cyst formation.
Psychosocial impact
- Quality of Life: The presence of multiple visible cysts can impact self-esteem and social interactions, especially during adolescence and early adulthood.
- Support: Counseling and support groups can be beneficial for individuals dealing with the psychological aspects of the condition.
Frequently Asked Questions
Steatocystoma Multiplex tends to be a life long condition. There are some reports of individuals developing these peculiar skin cysts even in old age. While treatments are good at removing individual cysts permanently, the cysts may appear on treated and non-treated areas over time. Fortunately, there has not been any cases where the cysts turned cancerous. It is generally a benign condition, and removal is often sought to improve quality of life and reduce distress.
No, steatocystoma multiplex is not life-threatening. However, it can impact the quality of life due to cosmetic concerns and potential discomfort from cysts.
The cysts in steatocystoma multiplex are benign and do not become cancerous. However, they can become inflamed or infected.
The presence of multiple visible cysts can affect self-esteem and social interactions, especially during adolescence and early adulthood. Regular monitoring and appropriate treatment can help manage these impacts.
If a cyst becomes painful or shows signs of infection (redness, swelling, pus), it is important to seek medical attention. A doctor can provide appropriate treatment, which may include antibiotics or drainage.
While lifestyle changes cannot cure the condition, maintaining good skin hygiene can help prevent secondary infections. Avoiding trauma to the cysts can reduce inflammation and discomfort.
The cost of treating steatocystoma multiplex can range from a few hundred to several thousand dollars, depending on the treatment approach and individual circumstances. It is advisable to consult with your doctor to discuss the most appropriate treatment options and obtain a detailed cost estimate based on the specific needs and available resources.
Steatocystoma Multiplex
Steatocystoma multiplex is a chronic genetic disorder characterized by multiple sebaceous cysts. While there is no cure, various treatments can manage symptoms and improve the quality of life for affected individuals. Regular dermatological care and support can help navigate the challenges posed by the condition.
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References:
Jamieson WA. Case of numerous cutaneous cysts scattered over the body. Edin Med J. 1873. 19:223-5.
Oh SW, Kim MY, Lee JS, Kim SC. Keratin 17 mutation in pachyonychia congenita type 2 patient with early onset steatocystoma multiplex and Hutchinson-like tooth deformity. J Dermatol. 2006 Mar. 33(3):161-4.
Cho S, Chang SE, Choi JH, Sung KJ, Moon KC, Koh JK. Clinical and histologic features of 64 cases of steatocystoma multiplex. J Dermatol. 2002 Mar. 29(3):152-6
Lee SJ, Choe YS, Park BC, Lee WJ, Kim do W. The vein hook successfully used for eradication of steatocystoma multiplex. Dermatologic Surgery. 2008. 33:82-84.
Bakkour W, Madan V. Carbon dioxide laser perforation and extirpation of steatocystoma multiplex. Dermatol Surg. 2014 Jun. 40(6):658-62.
