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syringoma

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syringoma

Posted by Ingrid on May 31, 2002 at 12:10:35:

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Syringoma

AUTHOR INFORMATION Section 1 of 9

Authored by Marcelo G Horenstein, MD, Director of Dermatopathology, Assistant Professor, Department of Pathology, University of South Alabama Medical Center

Coauthored by Christopher R Shea, MD, Head, Section of Dermatology, Professor, Department of Medicine, University of Chicago Medical Center

Marcelo G Horenstein, MD, is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, College of American Pathologists, Society for Investigative Dermatology, and United States and Canadian Academy of Pathology

Edited by Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School; Michael J Wells, MD, Staff Physician, Department of Dermatology, Texas Tech University Health Sciences Center; Edward F Chan, MD, Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; and Dirk M Elston, MD, Chairman, Department of Dermatology, Brooke Army-Wilford Hall Medical Center, Dermatology, Brooke Army Medical Center

Author's Email: Marcelo G Horenstein, MD
Editor's Email: Robert A Schwartz, MD, MPH

eMedicine Journal, May 3 2002, Volume 3, Number 5
INTRODUCTION Section 2 of 9

Background: Syringoma is a benign adnexal neoplasm formed by well-differentiated ductal elements. Its name is derived from the Greek word syrinx, which means pipe or tube.

Pathophysiology: Syringoma is a benign neoplasm that is traditionally considered to differentiate along eccrine lines. Many tumors that were traditionally thought to be eccrine have recently been shown to have apocrine differentiation.

Electron microscopy demonstrates ductal cells with numerous short microvilli, desmosomes, luminal tonofilaments, and lysosomes.

The histogenesis of syringomas may be related to apocrine elements or pluripotential stem cells.

Frequency:

In the US: Syringomas are fairly common lesions.
Internationally: Syringomas are fairly common lesions.
Mortality/Morbidity: These benign tumors are largely of cosmetic significance.

Sex: Females are affected more often than males.

Age: These tumors usually first appear at puberty; additional lesions can develop later. CLINICAL Section 3 of 9

History:

The lesions are asymptomatic.
Rarely, the patient may have a family history of similar lesions.
Physical:

Appearance of lesions
Syringomas are skin-colored or yellowish, small, dermal papules (see Image 1).
Sometimes, the lesions may appear translucent or cystic.
The surface can be rounded or flat-topped.
The lesions are usually smaller than 3 mm in diameter.
Distribution of lesions
The lesions are usually multiple, arranged in clusters, and symmetrically distributed.
Most commonly, lesions are limited to the upper parts of the cheeks and lower eyelids.
Other common sites include the axilla, chest, abdomen, penis, and vulva.
In the variant of eruptive syringoma, multiple lesions appear simultaneously, typically on the chest and lower abdomen.
Rarely, syringomas appear as unilateral linear nevoid lesions.
Differential diagnoses and related conditions
In rare instances, scalp syringomas can produce scarring alopecia.
On occasion, syringomas can be associated with milium cysts and vermiculate atrophoderma.
Clinically, syringomas on the face are must be distinguished from trichoepitheliomas and basal cell carcinomas.
Lesions on the eyelids may be confused with xanthelasma.
Eruptive syringomas on the trunk can resemble disseminated granuloma annulare.
Causes:

Syringomas are frequently incidental, although some familial cases may occur.
Eruptive syringomas (see Image 4) are more common in African Americans and Asians than in other patients.
Syringomas can be associated with Down syndrome.
Clear-cell syringomas may be associated with diabetes mellitus.
DIFFERENTIALS Section 4 of 9

Basal Cell Carcinoma
Granuloma Annulare
Trichoepithelioma

Other Problems to be Considered:

Milium cyst
Xanthelasma

WORKUP Section 5 of 9

Lab Studies:

Enzyme immunohistochemical tests demonstrate the presence of eccrine enzymes such as leucine aminopeptidase, succinic dehydrogenase, and phosphorylase.
The immunohistochemical pattern of cytokeratin expression indicates differentiation toward both the uppermost part of the dermal duct and the lower intra-epidermal duct (ie, sweat duct ridge). Distinguishing between eccrine and apocrine ducts may be difficult.
Histologic Findings: Syringoma is a tumor that is located mostly in the superficial dermis. It is composed of numerous small ducts embedded in a sclerotic stroma (see Image 2). The walls of the ducts are usually lined by 2 rows of cuboidal to flattened epithelial cells and contain a lumen filled with periodic acid-Schiff–positive, eosinophilic, amorphous debris. Some of the ducts have elongated tails of epithelial cells that produce the characteristic tadpole appearance. Keratinous cysts can be found on the surface. Rarely, tumor cells may appear clear as a result of glycogen accumulation (see Image 3).
The histologic differential diagnosis includes sclerosing (morphealike) basal cell carcinoma and trichoepithelioma. Importantly, syringoma should be distinguished from microcystic adnexal carcinoma, which has similar histologic features but tends to infiltrate the deep dermis and subcutaneous tissue. Chondroid syringoma is a different benign adnexal tumor with a characteristic myxoid cartilaginous matrix that is analogous to the mixed tumor (pleomorphic adenoma) of the salivary glands.

TREATMENT Section 6 of 9

Medical Care: Please see Surgical Care section below.

Surgical Care:

The main reason for treatment is cosmetic.
The goal of therapy should be the destruction of the tumor with minimal scarring and no recurrence.
Possible treatments include the following:
Excision
Electrodesiccation and curettage
Dermabrasion
Resurfacing with a carbon dioxide laser
FOLLOW-UP Section 7 of 9

Complications:

These benign tumors are largely of cosmetic significance.
Prognosis:

With treatment, the tumor should be destroyed with minimal scarring and no recurrence. See Surgical Care.
PICTURES Section 8 of 9

Caption: Picture 1. The multiple, small, yellow papules in the lower lid and upper part of the cheek correspond to syringomas. The blue cyst in the inner canthus is an eccrine hydrocystoma. Courtesy of Mark S. Brown, MD, University of South Alabama Medical Center.
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Caption: Picture 2. Histologic section of syringoma demonstrates numerous small ducts in a sclerotic stroma. Note the tadpole-shaped ducts.
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Caption: Picture 3. Histologic section of clear-cell syringoma.
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Caption: Picture 4. Eruptive syringomas.
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BIBLIOGRAPHY Section 9 of 9

Dupre A, Carrere S, Bonafe JL: Eruptive generalized syringomas, milium and atrophoderma vermiculata. Nicolau and Balus' syndrome. Dermatologica 1981; 162(4): 281-6[Medline].
Eckert F, Nilles M, Schmid U: Distribution of cytokeratin polypeptides in syringomas. An immunohistochemical study on paraffin-embedded material. Am J Dermatopathol 1992 Apr; 14(2): 115-21[Medline].
Shelley WB, Wood MG: Occult syringomas of scalp associated with progressive hair loss. Arch Dermatol 1980 Jul; 116(7): 843-4[Medline].
Urban CD, Cannon JR, Cole RD: Eruptive syringomas in Down's syndrome. Arch Dermatol 1981 Jun; 117(6): 374-5[Medline].
Wang JI, Roenigk HH Jr: Treatment of multiple facial syringomas with the carbon dioxide (CO2) laser. Dermatol Surg 1999 Feb; 25(2): 136-9[Medline].
Yung CW, Soltani K, Bernstein JE: Unilateral linear nevoidal syringoma. J Am Acad Dermatol 1981 Apr; 4(4): 412-6[Medline].

NOTE:
Medicine is a constantly changing science and not all therapies are clearly established. New research changes drug and treatment therapies daily. The authors, editors, and publisher of this journal have used their best efforts to provide information that is up-to-date and accurate and is generally accepted within medical standards at the time of publication. However, as medical science is constantly changing and human error is always possible, the authors, editors, and publisher or any other party involved with the publication of this article do not warrant the information in this article is accurate or complete, nor are they responsible for omissions or errors in the article or for the results of using this information. The reader should confirm the information in this article from other sources prior to use. In particular, all drug doses, indications, and contraindications should be confirmed in the package insert. FULL DISCLAIMER
eMedicine Journal, May 3 2002, Volume 3, Number 5

Syringoma excerpt

Follow Ups

Re: syringoma (Archive.) Walt Stoll 07:49:02 6/01/02 (0)

Re: syringoma (Archive.)

Posted by Walt Stoll on June 01, 2002 at 07:49:02:

In Reply to: syringoma posted by Ingrid on May 31, 2002 at 12:10:35:

Thanks, Ingrid.

Walt

Follow Ups:

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