Introduction
Background
Syringoma is a benign adnexal neoplasm formed by well-differentiated ductal elements. The name syringoma 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 been shown to have apocrine differentiation. Electron microscopy of syringomas 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.1
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
Syringomas are benign and are largely of cosmetic significance.
Sex
Females are affected by syringomas more often than males.
Age
Syringomas usually first appear at puberty; additional lesions can develop later.
Clinical
History
- Syringomas 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.2
- Syringomas may be associated with Down syndrome.3,4
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 of syringomas can be rounded or flat-topped.
- Syringomas are usually smaller than 3 mm in diameter.
- Eruptive syringomas commonly appear as hyperpigmented papules on the chest or penile shaft.
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.
Distribution of lesions
- Syringomas are usually multiple, arranged in clusters, and symmetrically distributed.
- Most commonly, syringomas are limited to the upper parts of the cheeks and lower eyelids.
- Other common sites for syringomas 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. The eruptive variant may involve the penis.5
- Rarely, syringomas appear as unilateral linear nevoid lesions.6
- In rare instances, scalp syringomas can produce scarring alopecia.7
- On occasion, syringomas can be associated with milia and vermiculate atrophoderma.8
- 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.
- Hamsch and Hartschuh suggest microcystic adnexal carcinoma can masquerade as syringoma.9
- Kao et al report that the lesions of Fox-Fordyce disease (multiple pruritic follicular papules) should be differentiated from syringomas.10
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.3,4
- Clear-cell syringomas may be associated with diabetes mellitus.11
Eruptive syringomas.
More on Syringoma |
 Overview: Syringoma |
| Differential Diagnoses & Workup: Syringoma |
| Treatment & Medication: Syringoma |
| Follow-up: Syringoma |
| Multimedia: Syringoma |
| References |
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References
Guitart J, Rosenbaum MM, Requena L. 'Eruptive syringoma': a misnomer for a reactive eccrine gland ductal proliferation?. J Cutan Pathol. Mar 2003;30(3):202-5. [Medline].
Uede K, Yamamoto Y, Furukawa F. Brooke-Spiegler syndrome associated with cylindroma, trichoepithelioma, spiradenoma, and syringoma. J Dermatol. Jan 2004;31(1):32-8. [Medline].
Schepis C, Siragusa M, Palazzo R, Ragusa RM, Massi G, Fabrizi G. Palpebral syringomas and Down's syndrome. Dermatology. 1994;189(3):248-50. [Medline].
Urban CD, Cannon JR, Cole RD. Eruptive syringomas in Down's syndrome. Arch Dermatol. Jun 1981;117(6):374-5. [Medline].
Petersson F, Mjornberg PA, Kazakov DV, Bisceglia M. Eruptive syringoma of the penis. A report of 2 cases and a review of the literature. Am J Dermatopathol. Jul 2009;31(5):436-8. [Medline].
Yung CW, Soltani K, Bernstein JE, Lorincz AL. Unilateral linear nevoidal syringoma. J Am Acad Dermatol. Apr 1981;4(4):412-6. [Medline].
Shelley WB, Wood MG. Occult syringomas of scalp associated with progressive hair loss. Arch Dermatol. Jul 1980;116(7):843-4. [Medline].
Dupre A, Carrere S, Bonafe JL, Christol B, Lassere J, Touron P. [Eruptive generalized syringomas, milium and atrophoderma vermiculata. Nicolau and Balus' syndrome (author's transl)]. Dermatologica. 1981;162(4):281-6. [Medline].
Hamsch C, Hartschuh W. Microcystic adnexal carcinoma - aggressive infiltrative tumor often with innocent clinical appearance. J Dtsch Dermatol Ges. Sep 25 2009;[Medline].
Kao PH, Hsu CK, Lee JY. Clinicopathological study of Fox-Fordyce disease. J Dermatol. Sep 2009;36(9):485-90. [Medline].
Jeon EK, Cho AY, Lee Y, Seo YJ, Park JK, Lee JH. Multiple yellow-brown papules on the neck and anterior trunk of a patient with diabetes mellitus. Clin Exp Dermatol. Jul 2009;34(5):643-4. [Medline].
Eckert F, Nilles M, Schmid U, Altmannsberger M. Distribution of cytokeratin polypeptides in syringomas. An immunohistochemical study on paraffin-embedded material. Am J Dermatopathol. Apr 1992;14(2):115-21. [Medline].
Al Aradi IK. Periorbital syringoma: a pilot study of the efficacy of low-voltage electrocoagulation. Dermatol Surg. Oct 2006;32(10):1244-50. [Medline].
Park HJ, Lee DY, Lee JH, Yang JM, Lee ES, Kim WS. The treatment of syringomas by CO(2) laser using a multiple-drilling method. Dermatol Surg. Mar 2007;33(3):310-3. [Medline].
Wang JI, Roenigk HH Jr. Treatment of multiple facial syringomas with the carbon dioxide (CO2) laser. Dermatol Surg. Feb 1999;25(2):136-9. [Medline].
Further Reading
Keywords
syringoma, benign adnexal neoplasms, syrinx, eruptive syringomas, clear cell syringomas, clear-cell syringomas
