Syringoma Clinical Presentation
- Author: Christopher R Shea, MD; Chief Editor: Dirk M Elston, MD more...
Syringomas are usually asymptomatic. However, rare cases have been associated with pruritus, especially in the setting of perspiration or when localized to the vulva. 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. Other associations include Nicolau-Balus syndrome, characterized by milia and atrophoderma vermiculatum, and Costello syndrome, characterized by various other cutaneous manifestations such as hyperkeratosis, hyperpigmentation, papillomas, and deep palmoplantar creases, as well as craniofacial, musculoskeletal, and neurologic abnormalities. In rare cases, syringoma can be associated with steatocystoma multiplex. The incidence of syringoma appears notably increased in association with Down syndrome.[7, 8, 9]
Syringomas are skin-colored or yellowish, generally small, dermal papules (see image below).
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. However, rare cases of giant syringoma have been reported. Eruptive syringomas typically appear as hyperpigmented papules on the chest, penile shaft, or vulva.
Syringomas are usually multiple, sometimes 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 dorsa of the hands. In the variant of eruptive syringoma, multiple lesions appear simultaneously, typically on the chest and lower abdomen. The eruptive variant may involve the penis and intertriginous areas. Rarely, syringomas appear as unilateral, linear, nevoid lesions.
Differential diagnoses and related conditions
In rare instances, scalp syringomas are associated with scarring alopecia.
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.
Syringomas are usually sporadic, but more than 10 cases of familial syringomas have been reported in the English literature. In the familial setting, syringomas tend to occur in preadolescence or adolescence, most commonly affect the face, and are usually inherited as an autosomal dominant trait.
Eruptive syringomas (see image below) are more common in African Americans and Asians than in other ethnic groups.
Syringoma can be associated with Down syndrome, in which case it is usually located in the periorbital region. However, rare cases of eruptive syringomas associated with Down syndrome have also been reported.[7, 8, 9]
Clear-cell syringoma may be associated with diabetes mellitus.
Hashimoto K, Gross BG, Lever WF. Syringoma. Histochemical and electron microscopic studies. J Invest Dermatol. 1966 Feb. 46(2):150-66. [Medline].
Guitart J, Rosenbaum MM, Requena L. Eruptive syringoma': a misnomer for a reactive eccrine gland ductal proliferation?. J Cutan Pathol. 2003 Mar. 30(3):202-5. [Medline].
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. 2009 Mar. 36(3):154-8. [Medline].
Uede K, Yamamoto Y, Furukawa F. Brooke-Spiegler syndrome associated with cylindroma, trichoepithelioma, spiradenoma, and syringoma. J Dermatol. 2004 Jan. 31(1):32-8. [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].
Nguyen V, Buka RL, Roberts BJ, Eichenfield LF. Cutaneous manifestations of Costello syndrome. Int J Dermatol. 2007 Jan. 46 (1):72-6. [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. 1981 Jun. 117(6):374-5. [Medline].
Ong GC, Lim KS, Chian LY. Eruptive syringoma in a patient with trisomy 21. Singapore Med J. 2010 Feb. 51(2):e46-7. [Medline].
Chan S, Millett C, Heymann WR. Giant syringomas: a case report. Cutis. 2012 Jan. 89(1):23-4. [Medline].
Koh MJ. Multiple acral syringomas involving the hands. Clin Exp Dermatol. 2009 Oct. 34(7):e438. [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. 2009 Jul. 31(5):436-8. [Medline].
Sakiyama M, Maeda M, Fujimoto N, Satoh T. Eruptive syringoma localized in intertriginous areas. J Dtsch Dermatol Ges. 2014 Jan. 12(1):72-3. [Medline].
Yung CW, Soltani K, Bernstein JE, Lorincz AL. Unilateral linear nevoidal syringoma. J Am Acad Dermatol. 1981 Apr. 4(4):412-6. [Medline].
Shelley WB, Wood MG. Occult syringomas of scalp associated with progressive hair loss. Arch Dermatol. 1980 Jul. 116(7):843-4. [Medline].
Hamsch C, Hartschuh W. Microcystic adnexal carcinoma - aggressive infiltrative tumor often with innocent clinical appearance. J Dtsch Dermatol Ges. 2009 Sep 25. [Medline].
Wallace JS, Bond JS, Seidel GD, Samie FH. An important mimicker: plaque-type syringoma mistakenly diagnosed as microcystic adnexal carcinoma. Dermatol Surg. 2014 Jul. 40(7):810-2. [Medline].
Kao PH, Hsu CK, Lee JY. Clinicopathological study of Fox-Fordyce disease. J Dermatol. 2009 Sep. 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. 2009 Jul. 34(5):643-4. [Medline].
Konstantinova AM, Hayes MM, Stewart CJ, Plaza JA, Michal M, Kerl K, et al. Syringomatous Structures in Extramammary Paget Disease: A Potential Diagnostic Pitfall. Am J Dermatopathol. 2016 Feb 9. [Medline].
Mitkov M, Balagula Y, Taube JM, Lockshin B. Plaque-like syringoma with involvement of deep reticular dermis. J Am Acad Dermatol. 2014 Nov. 71 (5):e206-7. [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. 1992 Apr. 14(2):115-21. [Medline].
Al Aradi IK. Periorbital syringoma: a pilot study of the efficacy of low-voltage electrocoagulation. Dermatol Surg. 2006 Oct. 32(10):1244-50. [Medline].
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. 2007 Mar. 33(3):310-3. [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].
Cho SB, Lee SH, Lee SJ, Lee JE, Kim DH. Syringoma treated with pinhole method. J Eur Acad Dermatol Venereol. 2009 Jul. 23(7):852-3. [Medline].
Cho SB, Kim HJ, Noh S, Lee SJ, Kim YK, Lee JH. Treatment of syringoma using an ablative 10,600-nm carbon dioxide fractional laser: a prospective analysis of 35 patients. Dermatol Surg. 2011 Apr. 37(4):433-8. [Medline].