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Dermatology > BENIGN NEOPLASMS
Steatocystoma Multiplex
Article Last Updated: May 24, 2006
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: Mary Bane, MD, Consulting Staff, Dermatologist Southwest Ohio
Mary Bane is a member of the following medical societies: American Academy of Dermatology, American Medical Association, and American Society for Dermatologic Surgery
Editors: Evan R Farmer, MD, Professor of Dermatology, Johns Hopkins University School of Medicine, Clinical Professor of Pathology, Virginia Commonwealth University School of Medicine; Consulting Staff, Department of Dermatology, Johns Hopkins Hospital, VCU Health Services; Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA; Warren R Heymann, MD, Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey; Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
Author and Editor Disclosure
Synonyms and related keywords:
sebocystomatosis, hereditary epidermal polycystic disease
Background
Steatocystoma multiplex (SM) is an uncommon autosomal dominant disorder involving the pilosebaceous unit. This condition (described by Jamieson in 1873) is characterized by the development of numerous sebum-containing dermal cysts. The cysts can be widespread and difficult to treat.
Steatocystoma simplex is the noninheritable solitary tumor counterpart to SM.
Pathophysiology
SM is a familial disorder of the pilosebaceous unit that has an autosomal dominant transmission. The lesions are a nevoid formation of abortive hair follicles at the site where sebaceous glands attach. Electron microscopy studies demonstrate cyst wall cells undergoing trichilemmal keratinization similar to that of the isthmus portion of the outer hair sheath. The relationship of SM to the development of sebaceous glands and its presentation at puberty suggest a hormonal trigger for lesion growth. This also corresponds with the common distribution of cysts on the upper-central torso.
SM frequently is associated with vellus hair cysts (VHC) in the same patient. These 2 cyst types present with the same distribution and timing and may represent points along a continuum on which some include trichostasis spinulosa. SM also has been seen in patients with pachyonychia congenita type 2 (PC-2), in which a keratin 17 (K17) mutation was localized. This mutation also has been reported in SM cases without the features of PC-2. In SM associated with VHC, no K17 mutation has been found. SM may be a heterogeneous group of genotypes that have similar phenotypic or clinical presentations.
Frequency
United States
SM is considered rare; the true incidence is unknown.
Mortality/Morbidity
SM is a benign disorder. In some patients, it may have psychosocial implications resulting from the disfigurement of widespread lesions or from scarring seen in the inflammatory variant steatocystoma suppurativa.
Race
No racial predilection has been found.
Sex
Both sexes are equally affected.
Age
Cysts present during adolescence and early adulthood. SM is a lifelong condition.
History
Affected individuals often present with the complaint of an increasing number of smooth flesh-to-yellow colored cysts. The cysts usually are asymptomatic but occasionally rupture into the dermis, become inflamed, and heal by scarring. For this reason, the presenting complaint can be misdiagnosed as scarring acne.
Physical
- Cysts are concentrated in areas where high numbers of sebaceous glands are found (upper torso, proximal extremities).
- Cysts can present anywhere on the skin (ie, face, groin).
- The involved area becomes studded with deep, flesh-to-yellow colored, superficial, dermal papules.
- Some lesions may become inflamed, and erythema may surround them.
- When a cyst spontaneously ruptures to the skin surface or is incised and drained intentionally, the contents appear as an odorless creamy or oily fluid.
Causes
SM is a familial disorder of the pilosebaceous unit that has an autosomal dominant transmission.
Acne Vulgaris
Eruptive Vellus Hair Cysts
Follicular Infundibulum Tumor
Gardner Syndrome
Milia
Syringoma
Histologic Findings
Cysts are located in the mid dermis. The cyst lining is a crenulated or wavy, homogeneous, eosinophilic horny layer collapsed around thin cystic spaces. The spaces hold varying amounts of keratin, vellus hairs, and sebum esters, the latter of which often are removed by tissue processing. Walls are formed from several layers of epithelial cells with embedded flattened lobules of sebaceous glands among the epithelial cells. Invaginations resembling hair follicles also can be found emptying into the cyst. Each cyst unit is attached to the overlying normal epidermis by a thin strand of undifferentiated epithelial cells.
Medical Care
Treatment usually is not required for this benign condition; however, treatment has been attempted with variable results.
- Steatocystoma suppurativa: Treatment is indicated for this scarring inflammatory version of the disorder and involves oral antibiotics from the tetracycline class. Isotretinoin therapy also has been effective in some patients, but in others, isotretinoin has caused the condition to flare.
- Cosmesis: The patient may desire intervention for cosmetic reasons. Unfortunately, isotretinoin (despite known effects of decreasing sebaceous gland activity) has shown inconsistent results, according to the literature. In at least 1 patient, isotretinoin appeared to worsen the condition, which necessitated cessation of the drug.
Surgical Care
- Aspiration: Individual cyst contents can be aspirated through an 18-gauge needle, but the effect is only temporary. This technique is thought to be the treatment of choice for facial lesions because the scarring associated with excisional approaches is avoided.
- Surgical excision: Excision of all cysts on any 1 patient is not feasible because of the large numbers of cysts usually present. However, excision of some of the larger lesions can be performed using a small surgical punch or a sharp-tipped cautery point to puncture the cyst wall. The cyst's contents then can be expressed prior to everting the cyst lining with forceps to destroy it by either clip removal or electrodesiccation. The small punch site is allowed to heal by secondary intention.
- Carbon dioxide laser: At least 1 published report exists of the successful treatment of SM using a carbon dioxide laser.
The goals of pharmacotherapy are to reduce morbidity and to prevent complications.
Drug Category: Antibiotics
Tetracycline derivatives with their anti-inflammatory side effects have been helpful in treating steatocystoma suppurativa.
| Drug Name | Tetracycline (Sumycin) |
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| Description | Treats gram-positive and gram-negative organisms, and mycoplasmal, chlamydial, and rickettsial infections. Inhibits bacterial protein synthesis by binding with 30S and possibly 50S ribosomal subunit(s). Also is useful for its anti-inflammatory effects. |
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| Adult Dose | 500 mg PO bid |
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| Pediatric Dose | <8 years: Not recommended
>8 years: 250-500 mg PO bid |
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| Contraindications | Documented hypersensitivity; severe hepatic dysfunction |
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| Interactions | Bioavailability decreases with antacids containing aluminum, calcium, magnesium, iron, or bismuth subsalicylate; can increase hypoprothrombinemic effects of anticoagulants |
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| Pregnancy | D - Unsafe in pregnancy
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| Precautions | Photosensitivity may occur with prolonged exposure to sunlight or tanning equipment; reduce dose in renal impairment; tetracycline use during tooth development (last one half of pregnancy through age 8 y) can cause permanent discoloration of teeth; Fanconilike syndrome may occur with outdated tetracyclines |
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Drug Category: Retinoids
Decrease size of sebaceous glands and decrease their sebum production. Retinoids also have anti-inflammatory effects by decreasing production of certain leukotrienes.
| Drug Name | Isotretinoin (Accutane) |
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| Description | Oral agent that treats serious dermatologic conditions. Synthetic 13-cis isomer of naturally occurring tretinoin (trans-retinoic acid). Both agents are related structurally to beta-carotene. Decreases sebaceous gland size and sebum production. May inhibit sebaceous gland differentiation and abnormal keratinization.
Only those physicians experienced or trained in use should prescribe. |
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| Adult Dose | 0.5-1.5 mg/kg/d 2-20 mo (latter duration was experimental protocol) |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; sexually active without birth control, significant hepatic dysfunction, or severely elevated triglycerides |
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| Interactions | Toxicity may occur with beta carotene coadministration; pseudotumor cerebri or papilledema may occur when coadministered with tetracyclines; may reduce plasma levels of carbamazepine; increased risk of hepatotoxicity with MTX |
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| Pregnancy | X - Contraindicated in pregnancy
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| Precautions | Ensure that female patients are not pregnant prior to starting isotretinoin. HCG in female patients should be checked monthly; may decrease night vision; may be associated with development of hepatitis; occasional exaggerated healing response of acne lesions (excessive granulation with crusting) may occur; diabetes patients may experience problems controlling blood sugar; reduce exposure to UV light or sunlight until tolerance achieved. Although there have been some reports of depression and suicide associated with isotretinoin use, this remains controversial since studies do not show an increase of either in the isotretinoin group compared to the general population. |
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Prognosis
- Prognosis for patients with SM is excellent. No reports exist of malignant transformation within these benign adnexal tumors.
Medical/Legal Pitfalls
- SM is a benign disorder and carries little risk of medicolegal liability.
| Media file 1:
Steatocystoma multiplex on the chest of an adolescent female. |
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| Media file 2:
Steatocystoma multiplex with typical-appearing smooth yellow and white dermal cysts. |
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| Media file 3:
Note the crenulated eosinophilic lining of the cyst wall (10X magnification). |
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Media type: Photo
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| Media file 4:
Note the sebaceous glands within the cyst wall (2X scanning view). |
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Media type: Photo
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- Brownstein MH. Steatocystoma simplex. A solitary steatocystoma. Arch Dermatol. Jun 1982;118(6):409-11. [Medline].
- Corden LD, McLean WH. Human keratin diseases: hereditary fragility of specific epithelial tissues. Exp Dermatol. Dec 1996;5(6):297-307. [Medline].
- Jamieson WA. Case of numerous cutaneous cysts scattered over the body. Edin Med J. 1873;19:223-5.
- Kligman AM, Kirschbaum JD. Steatocystoma multiplex: a dermoid tumor. J Invest Dermatol. 1964;42:383-7.
- Krahenbuhl A, Eichmann A, Pfaltz M. CO2 laser therapy for steatocystoma multiplex. Dermatologica. 1991;183(4):294-6. [Medline].
- Plewig G, Wolff HH, Braun-Falco O. Steatocystoma multiplex: anatomic reevaluation, electron microscopy, and autoradiography. Arch Dermatol Res. 1982;272(3-4):363-80. [Medline].
- Rook A, Wilkinson DS, Ebling FJG. Rook/Wilkinson/Ebling Textbook of Dermatology. Vol 2, 3. Blackwell Science, Inc;1998:1476, 1666-7, 1983.
- Smith FJ, Corden LD, Rugg EL, et al. Missense mutations in keratin 17 cause either pachyonychia congenita type 2 or a phenotype resembling steatocystoma multiplex. J Invest Dermatol. Feb 1997;108(2):220-3. [Medline].
Steatocystoma Multiplex excerpt Article Last Updated: May 24, 2006
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