Syringoma Clinical Presentation

  • Author: Christopher R Shea, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Aug 17, 2010
 

History

Syringomas are asymptomatic. However, in some rare cases, they have been associated with pruritus, especially in the setting of perspiration.[2] Uncommonly, the patient may have a family history of similar lesions. Rarely, syringomas may be associated with the Brooke-Spiegler syndrome, an autosomal dominant disease characterized by the development of multiple cylindromas, trichoepitheliomas, and occasional spiradenomas.[3] Additionally, syringomas may be associated with Down syndrome.[4, 5, 6]

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Physical

The appearance of syringomas is as follows:

  • Syringomas are skin-colored or yellowish, small, dermal papules (see image below).The multiple, small, yellow papules in the lower lThe 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 hidrocystoma. Courtesy of Mark S. Brown, MD, University of South Alabama Medical Center.
  • Sometimes, the lesions may appear translucent or cystic.
  • The surface of syringomas can be rounded or flat.
  • Syringomas are usually smaller than 3 mm in diameter.
  • Eruptive syringomas typically appear as hyperpigmented papules on the chest or penile shaft.

The distribution of syringomas is as follows:

  • 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 characteristic sites for syringomas include the axilla, chest, abdomen, penis, and vulva.
  • Case reports have described syringomas limited to the dorsum of the hands.[7]
  • In the variant of eruptive syringoma, multiple lesions appear simultaneously, typically on the chest and lower abdomen. The eruptive variant may involve the penis.[8]
  • Rarely, syringomas appear as unilateral, linear, nevoid lesions.[9]

Differential diagnoses and related conditions are as follows:

  • In rare instances, scalp syringomas can produce scarring alopecia.[10]
  • On occasion, syringomas can be associated with milia and vermiculate atrophoderma.[11]
  • In rare cases, syringoma can be associated with steatocystoma multiplex.[2]
  • Clinically, syringomas on the face must be distinguished from trichoepitheliomas and basal cell carcinomas.
  • Lesions on the eyelids may be confused with xanthelasmas.
  • Eruptive syringomas on the trunk can resemble disseminated granuloma annulare.
  • Microcystic adnexal carcinoma can masquerade as syringoma.[12]
  • Lesions of Fox-Fordyce disease (multiple pruritic follicular papules) should be differentiated from syringomas.[13]
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Causes

Syringomas are frequently incidental, although some familial cases may occur. More than 10 English language cases of familial syringomas have been reported. They tend to occur in preadolescence or adolescence, have no sexual predilection, most commonly involve the face, and are inherited as autosomal dominant traits or as a result of germline or somatic mutations.[2]

Eruptive syringomas (see image below) are more common in African Americans and Asians than in other ethnic groups.

Eruptive syringomas. Eruptive syringomas.

Syringomas can be associated with Down syndrome, in which case they are usually located in the periorbital region. However, rare cases of eruptive syringomas associated with Down syndrome have also been reported.[4, 5, 6]

Clear-cell syringomas may be associated with diabetes mellitus.[14]

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Contributor Information and Disclosures
Author

Christopher R Shea, MD  Professor and Chief, Section of Dermatology, Department of Medicine, University of Chicago

Christopher R Shea, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society of Dermatopathology, Arthur Purdy Stout Society, Association of Professors of Dermatology, Chicago Dermatological Society, Dermatology Foundation, Illinois Dermatological Society, International Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Ibiayi Dagogo-Jack  University of Chicago Pritzker School of Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Edward F Chan, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

  1. 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].

  2. Marzano AV, Fiorani R, Girgenti V, Crosti C, Alessi E. Familial syringoma: report of two cases with a published work review and the unique association with steatocystoma multiplex. J Dermatol. Mar 2009;36(3):154-8. [Medline].

  3. 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].

  4. 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].

  5. Urban CD, Cannon JR, Cole RD. Eruptive syringomas in Down's syndrome. Arch Dermatol. Jun 1981;117(6):374-5. [Medline].

  6. Ong GC, Lim KS, Chian LY. Eruptive syringoma in a patient with trisomy 21. Singapore Med J. Feb 2010;51(2):e46-7. [Medline].

  7. Koh MJ. Multiple acral syringomas involving the hands. Clin Exp Dermatol. Oct 2009;34(7):e438. [Medline].

  8. 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].

  9. Yung CW, Soltani K, Bernstein JE, Lorincz AL. Unilateral linear nevoidal syringoma. J Am Acad Dermatol. Apr 1981;4(4):412-6. [Medline].

  10. Shelley WB, Wood MG. Occult syringomas of scalp associated with progressive hair loss. Arch Dermatol. Jul 1980;116(7):843-4. [Medline].

  11. 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].

  12. Hamsch C, Hartschuh W. Microcystic adnexal carcinoma - aggressive infiltrative tumor often with innocent clinical appearance. J Dtsch Dermatol Ges. Sep 25 2009;[Medline].

  13. Kao PH, Hsu CK, Lee JY. Clinicopathological study of Fox-Fordyce disease. J Dermatol. Sep 2009;36(9):485-90. [Medline].

  14. 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].

  15. 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].

  16. Jamalipour M, Heidarpour M, Rajabi P. Generalized eruptive syringomas. Indian J Dermatol. 2009;54(1):65-7. [Medline]. [Full Text].

  17. Al Aradi IK. Periorbital syringoma: a pilot study of the efficacy of low-voltage electrocoagulation. Dermatol Surg. Oct 2006;32(10):1244-50. [Medline].

  18. Park HJ, Lee DY, Lee JH, Yang JM, Lee ES, Kim WS. The treatment of syringomas by CO2 laser using a multiple-drilling method. Dermatol Surg. Mar 2007;33(3):310-3. [Medline].

  19. 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].

  20. Cho SB, Lee SH, Lee SJ, Lee JE, Kim DH. Syringoma treated with pinhole method. J Eur Acad Dermatol Venereol. Jul 2009;23(7):852-3. [Medline].

 
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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 hidrocystoma. Courtesy of Mark S. Brown, MD, University of South Alabama Medical Center.
Eruptive syringomas.
Histologic section of syringoma demonstrates numerous small ducts in a sclerotic stroma. Note the tadpole-shaped ducts.
Histologic section of clear-cell syringoma.
 
 
 
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