Introduction
Steatocystoma Multiplex stands out as an intriguing and enigmatic ailment within the large field of dermatological conditions. This disorder, which is characterized by the development of multiple benign cysts beneath the surface of the skin, poses medical and aesthetic difficulties for those who are affected. Steatocystoma Multiplex has attracted medical experts and researchers despite being relatively uncommon, leading to efforts to comprehend its underlying origins, diagnosis, and therapy alternatives.
This article explores the mysterious realm of steatocystoma multiplex, learning about its causes, clinical symptoms, and effects on people who are affected, navigating through the complex network of information surrounding this ailment, and shedding light on both its distinctive features and the management of the condition.
What Is Steatocystoma Multiplex?
Steatocystoma multiplex is a rare skin condition characterized by the development of multiple cutaneous cysts that are benign or noncancerous in nature. It is also referred to as a nevoid malformation (a malformation that resembles a nevus or mole) of the pilosebaceous unit (the collective complex of the hair shaft, hair follicle, and sebaceous gland). The cysts may be distributed across the body and filled with sebum (an oily substance that lubricates the hair and skin). This is because these cysts develop and grow from the sebaceous glands in the skin.
Steatocystoma multiplex is typically inherited, while rare cases may be non-hereditary or sporadic in nature. The inheritance of steatocystoma multiplex follows an autosomal dominant pattern. This means that a single copy of the mutated gene (in this case, the KRT17 gene) inherited from one parent is enough to cause the condition. Non-hereditary cases will generally have no family history of the condition.
Some of the other terms that may be used to refer to steatocystoma multiplex include the following:
- Multiplex steatocystoma.
- Multiple sebaceous cysts.
- Sebocystomatosis.
Who Is Affected by Steatocystoma Multiplex and What Is the Prevalence?
Knowledge of the prevalence of steatocystoma multiplex is pretty limited, but it is purported to be a rare condition. For steatocystoma multiplex to be inherited, it can be reasonably expected that one parent will have the condition. This means that the people most likely to be affected are those with a family history of the disease (typically a parent). The age of onset of the disease is around 26 years (age range- four to 64 years). Both males and females can get the condition, but females are slightly more likely to be affected than males. The female-to-male ratio is about 1.2:1.
What Are the Causes of Steatocystoma Multiplex?
Steatocystoma multiplex may typically be inherited or rarely be sporadic (non-inherited). The familial steatocystoma multiplex condition is caused due to heterozygous mutations in the KRT17 gene on chromosome 17q21. The KRT17 gene is responsible for the production of the keratin 17 protein produced in the hair follicles, skin on the palms and soles, and nails. It is believed that steatocystomas arise from the abnormal lining of the sebaceous ducts (passages to oil glands).
Mutations in the KRT17 gene cause structural changes in keratin 17. This results in a lack of stable network formation in cells. The destruction of stable cell networks essentially means that the growth of the cells in the nails and the skin are interrupted or disrupted. The cells in sebaceous glands are also disrupted, forming the characteristic sebum-containing cysts called steatocystoma multiplex. In sporadic or non-inherited cases of steatocystoma multiplex, the cause is unknown. No mutation is seen in the KRT17 gene.
What Are the Signs and Symptoms of Steatocystoma Multiplex?
Steatocystoma multiplex usually presents as mobile, non-tender cysts of the dermis that show no visible changes in the epidermis. These cysts are small (between 2 mm and 20 mm) but may also go up to a few centimeters. They may be round or oval, soft or firm, and semi-translucent raised lesions that contain sebum (a yellowish, oily liquid). A few hairs may be present on the lesion, called eruptive vellus hair cysts. Occasionally, an identifiable punctum may be centrally seen (a dome-shaped projection from which the sebum can be squeezed out).
The sites most commonly affected by these lesions include the following:
- Chest.
- Groin.
- Axillae or armpits.
- Upper arms.
- Abdomen.
- Face.
- Rarely, there may also be cystic development all over the body.
- Some cases have presented as harmless cysts of the scalp called wens from which the fatty secretion obtained from sebaceous glands may be identified or elicited.
The condition has no known or recognizable associated internal signs or symptoms. While the cysts are generally asymptomatic, a suppurative variant of steatocystoma multiplex has been identified where cysts may become inflamed and suppurate even with mild trauma. Sporadic cases of steatocystoma multiplex have been presented with isolated cysts in the vulva and scrotum late in life and are unassociated with genetic mutations.
How Is Steatocystoma Multiplex Diagnosed?
The diagnosis of steatocystoma multiplex involves the use of a skin biopsy. The typical presentation with a skin biopsy is a dermal cyst with thin walls and a sebaceous gland within these walls. Lanugo hair may be noted in the lumen of the cyst.
The differential diagnoses for this disease include the following conditions:
- Epidermoid cysts.
- Milia.
- Acne conglobata.
How Is Steatocystoma Multiplex Treated?
Treatment options for steatocystoma multiplex include the following options:
- Cryosurgery.
- Surgical removal.
- Aspiration.
- Laser therapy.
- Electrosurgery.
- Most individual cysts may be surgically removed. Incisions in the skin can help remove the cyst and the contents of the cyst. In cases where the cyst is firmly attached to the skin, an excision biopsy can be considered.
- The use of oral Isotretinoin helps reduce the size of the cysts as well as inflammation.
- Oral antibiotics may also be used to reduce the inflammation seen.
- The suppurative variant may require incision and drainage of the cysts followed by antimicrobial therapy.
- Advanced options have included the use of radiofrequency devices to make incisions and express the cyst. This is followed by cyst wall removal using forceps. The advantages of this technique include the nonnecessity of sutures, the lack of scarring, and the absence of recurrence even five months post-treatment.
- Lasers have also been used to target both the superficial sebaceous glands and the cystic portions, with good cosmetic results (no scarring) and no recurrence.
Conclusion:
While steatocystoma multiplex is rare and mostly harmless, the associated psychosocial impact may be quite disruptive to everyday life. However, the availability of a range of treatment options with excellent results and often no lasting cosmetic or esthetic issues has meant that this condition is not something to worry about. With the right treatment, good supportive care, and mental healthcare support, patients can lead a happy, healthy, and entirely embarrassment-free life.
