Description, Causes and Risk Factors:
Widespread, multiple, thin-walled cysts of the skin that are lined by squamous epithelium, including lobules of sebaceous cells.
Steatocystoma multiplex is an epidermal polycystic disease, usually begins in late childhood and persists indefinitely. Steatocystoma multiplex is an autosomal dominant disorder; however, in some cases no familial pattern can be established. It is a very uncommon condition, usually beginning in adolescence or early adult life with equal sex distribution.
Steatocystoma multiplex can be caused by mutations in the KRT17 gene. This gene provides instructions for making a protein called keratin 17, which is produced in the nails, the hair follicles, and the skin on the palms of the hands and soles of the feet. It is also found in the skin's sebaceous glands. Keratin 17 partners with a similar protein called keratin 6b to form networks that provide strength and resilience to the skin, nails, and other tissues.
The KRT17 gene mutations that cause steatocystoma multiplex alter the structure of keratin 17, preventing it from forming strong, stable networks within cells. The defective keratin network disrupts the growth and function of cells in the skin and nails, including cells that make up the sebaceous glands. These abnormalities lead to the growth of sebum-containing cysts in people with steatocystoma multiplex. However, it is unclear why steatocystomas are typically the only feature of this disorder. In some cases, people with steatocystoma multiplex do not have an identified mutation in the KRT17 gene. The cause of the condition in these individuals is unknown.
Although the prevalence of steatocystoma multiplex is unknown, it appears to be rare.
A patient with steatocystoma multiplex was treated with 0.75 mg per kg per day of oral isotretinoin. After one month of minimal improvement the patient discontinued therapy. Over the next two months he noted some shrinkage of his pre-existing lesions, which persisted for a follow-up period of six months. Isotretinoin may show a beneficial response in some cases of steatocystoma multiplex even after use of the drug is discontinued. Isotretinoin is known to have effects beyond its treatment period that are related to its long serum half-life. A small number of patients with steatocystoma multiplex have been treated with isotretinoin, with varied responses. This is the first case of a patient who discontinued therapy before a response occurred and then showed a response later.
Clinically, it is characterized by multiple smooth, firm, dermal cystic papules and nodules within the dermis, varying in diameter from a few millimeters to twenty millimeters or more. They usually appear or become larger at puberty. The trunk, scrotum, and the proximal part of the limbs are most commonly involved, particularly presternal area. No punctum is usually apparent over cyst. The deeper lesions are skin-colored and superficial lesions may have a yellowish color. When punctured, the cysts discharge a characteristic oily or creamy fluid. The lesions are usually asymptomatic, while some may become inflamed, suppurate, and heal with scarring. Familial clustering and congenital forms of steatocystoma multiplex have been reported.
The diagnosis is usually established clinically, although biopsy may be required. Only a few case reports have been published that detail the radiologic features of pathologically proven steatocystoma multiplex.
The histopathologic findings of steatocystoma multiplex are multiple round cysts in the mid and deep dermis and cyst walls composed of three to five layers of squamous epithelium lined with flattened sebaceous glands and undulating acellular eosinophilic cuticles. Steatocystoma multiplex seems to arise in the sebaceous ducts. The cysts contain a clear oily liquid, small amounts of keratinous material.
The condition poses no threat to a patient's health but is frequently a cosmetic problem. There are few satisfactory treatment options.
Aspiration: Simple aspiration with an 18-gauge needle has been used successfully in minimizing scarring of facial lesions. Although postoperative scars are minimal, a high rate of recurrence has been observed. A variation of this method by insertion and gentle extirpation of cystic contents without removing the cyst wall is thought to be the treatment of choice in the management of facial lesions and those smaller than 1.5 cm in diameter.
Surgical excision: Traditionally, surgery with elliptical excisions, flaps, or grafts was the most commonly practiced method. But often it is impractical for widespread lesions and has fallen out of favor due to its time-consuming nature and the associated risk of scarring. Punch excision followed by cyst removal has also been used in the past.
Incisional treatments: There are several type of incisional treatments. A number 11 surgical blade was used to produce a mini-incision followed by expression of cyst contents and excochleation of the cyst wall using a 1-mm curette. This resulted in minimal scarring and a low rate of recurrence. A modified surgical technique, used on more than 50 lesions, is incision with a sharp-tipped cautery followed by expression of cyst contents and forceps-assisted removal of the cyst wall. This technique resulted in minimal depressed scarring and slight hypopigmentation with no evidence of recurrence. A phlebectomy hook may also be used to produce small incisions, 2-3 mm in length, followed by a removal of the cyst wall.
Carbon dioxide laser: The advent of carbon dioxide laser has allowed treatment of multiple lesions during a single treatment session, with no anesthesia, a low percentage of recurrence, and good cosmesis.
Cryosurgery: Cryosurgery has been used in the past but residual scarring is a limitation.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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