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Dermatology > BENIGN NEOPLASMS
Syringoma
Article Last Updated: May 23, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 9  
Author: Marcelo G Horenstein, MD, Director of Dermatopathology, The Dermatology Group
Marcelo G Horenstein 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
Coauthor(s):
Christopher R Shea, MD, Professor and Chief, Section of Dermatology, Department of Medicine, University of Chicago
Editors: Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School; Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center; Edward F Chan, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Author and Editor Disclosure
Synonyms and related keywords:
benign adnexal neoplasms, syrinx, eruptive syringomas, clear cell syringomas, clear-cell syringomas
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, which is traditionally considered to differentiate along eccrine lines. 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 intraepidermal duct (ie, sweat duct ridge). However, sometimes distinguishing between eccrine and apocrine ducts is difficult and 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 is most likely related to eccrine elements or pluripotential stem cells. Some investigators have suggested that cases of eruptive syringoma may represent a hyperplastic response of the eccrine duct to an inflammatory reaction rather than a true adnexal neoplasm. In this setting, they propose the term syringomatous dermatitis for such cases. Likewise, the scalp "syringomas" seen in scarring alopecia represent a reactive proliferation in response to the fibrosis.
Frequency
International
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.
History
- The lesions are asymptomatic.
- Rarely, the patient may have a family history of similar lesions. Rarely, syringomas may be associated with the Brooke-Spiegler syndrome, an autosomally dominant disease characterized by the development of multiple cylindromas, trichoepitheliomas, and occasional spiradenomas.
- Syringomas may be associated with Down syndrome.
Physical
Appearance of lesions - Syringomas are skin-colored or yellowish, small, dermal papules (see Media File 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.
- Eruptive syringomas commonly appear as hyperpigmented papules on the chest or penile shaft.
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 milia 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 Media File 2) 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.
Acne Vulgaris
Apocrine Hidrocystoma
Basal Cell Carcinoma
Colloid Milium
Cowden Disease (Multiple Hamartoma Syndrome)
Cutaneous Tuberculosis
Favre-Racouchot Syndrome (Nodular Elastosis with Cysts and Comedones)
Granuloma Annulare
Microcystic Adnexal Carcinoma
Microcystic Adnexal Carcinoma
Milia
Sebaceous Hyperplasia
Steatocystoma Multiplex
Trichoepithelioma
Other Problems to be Considered
Xanthelasma
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 Media File 3). 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 Media File 4). The histologic differential diagnosis includes sclerosing (morphealike) basal cell carcinoma and desmoplastic 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. Therefore, partial superficial biopsies may be problematic in this situation.
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.
Medical Care
Please see Surgical Care section below.
Surgical Care
- The main reason for treatment is cosmetic, in particular, for syringomas of the eyelids and cheeks, which may be conspicuous.
- The goal of therapy should be the destruction of the tumor with minimal scarring and no recurrence.
- No comparative studies and no long-term follow-up studies are available on which to base definitive recommendations for treatment. Possible treatments include the following:
- Surgical excision with primary suturing
- Scissor excision with secondary intention healing
- Electrocautery
- Electrodesiccation and curettage
- Carbon dioxide or Er:YAG laser ablation
- Cryotherapy
- Dermabrasion
- Trichloroacetic acid
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.
| Media file 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. |
 |  View Full Size Image | |
Media type: Photo
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| Media file 3:
Histologic section of syringoma demonstrates numerous small ducts in a sclerotic stroma. Note the tadpole-shaped ducts. |
 | View Full Size Image | |
Media type: Photo
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Syringoma excerpt Article Last Updated: May 23, 2007
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