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==Pathophysiology==
==Pathophysiology==
===Pathogenesis===
===Pathogenesis===
The pathogenesis of syringoma is characterized by a hyperplastic response of the eccrine duct to an inflammatory reaction rather than a true adnexal neoplasm. Some authors have proposed the term "syringomatous dermatitis" for such cases. The scalp "syringomas" seen in scarring alopecia may represent a reactive proliferation in response to the fibrosis.
The pathogenesis of syringoma is characterized by a hyperplastic response of the eccrine duct to an inflammatory reaction rather than a true [[adnexal]] neoplasm. Some authors have proposed the term "syringomatous dermatitis" for such cases. The scalp "syringomas" seen in scarring [[alopecia]] may represent a reactive proliferation in response to the fibrosis.
It is hypothesized that phosphorylase deficiency, resulting from elevated glucose levels seen in diabetes leads to an accumulation of glycogen in the skin and within the clear cells.<ref name="pmid4286059">{{cite journal| author=Hashimoto K, Gross BG, Lever WF| title=Syringoma. Histochemical and electron microscopic studies. | journal=J Invest Dermatol | year= 1966 | volume= 46 | issue= 2 | pages= 150-66 | pmid=4286059 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4286059  }} </ref><ref name="pmid12641781">{{cite journal| author=Guitart J, Rosenbaum MM, Requena L| title='Eruptive syringoma': a misnomer for a reactive eccrine gland ductal proliferation? | journal=J Cutan Pathol | year= 2003 | volume= 30 | issue= 3 | pages= 202-5 | pmid=12641781 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12641781  }} </ref>*Syringoma is a benign neoplasm that differentiates along eccrine lines.
It is hypothesized that [[phosphorylase deficiency]], resulting from elevated glucose levels seen in [[diabetes]] leads to an accumulation of [[glycogen]] in the skin and within the clear cells.<ref name="pmid4286059">{{cite journal| author=Hashimoto K, Gross BG, Lever WF| title=Syringoma. Histochemical and electron microscopic studies. | journal=J Invest Dermatol | year= 1966 | volume= 46 | issue= 2 | pages= 150-66 | pmid=4286059 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4286059  }} </ref><ref name="pmid12641781">{{cite journal| author=Guitart J, Rosenbaum MM, Requena L| title='Eruptive syringoma': a misnomer for a reactive eccrine gland ductal proliferation? | journal=J Cutan Pathol | year= 2003 | volume= 30 | issue= 3 | pages= 202-5 | pmid=12641781 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12641781  }} </ref>*Syringoma is a benign neoplasm that differentiates along eccrine lines.
*The pathophysiology of syringomas is largely unknown;however, the scientists propose that syringomas are benign growths that arise from the intraepidermal portion of eccrine ducts. It has also been suggested that a hamartoma of pluripotent stem cells could precede the pathological process of eruptive syringomas. Syringomas may also be under hormonal influence which may explain its female predominance.
*The pathophysiology of syringomas is largely unknown;however, the scientists propose that syringomas are benign growths that arise from the intraepidermal portion of [[eccrine]] ducts. It has also been suggested that a [[hamartoma]] of pluripotent [[stem cells]] could precede the pathological process of eruptive syringomas. Syringomas may also be under hormonal influence which may explain its female predominance.
*The mutation in chromosome 16q22 has been associated with the development of familial autosomal dominant syringoma.
*The mutation in chromosome 16q22 has been associated with the development of familial [[autosomal dominant]] syringoma.
===Associated Conditions===
===Associated Conditions===
*Syringomas can be found in association with other symptoms as part of a syndrome such as:
*Syringomas can be found in association with other symptoms as part of a syndrome such as:
Line 54: Line 54:


===Microscopic Pathology===
===Microscopic Pathology===
*On immunohistochemical tests, the presence of eccrine enzymes such as leucine aminopeptidase, succinic dehydrogenase, and phosphorylase and the immunohistochemical pattern of cytokeratin expression which indicates differentiation toward both the uppermost part of the dermal duct and the lower intraepidermal duct are characteristic findings of syringoma.
*On immunohistochemical tests, the presence of [[eccrine]] enzymes such as [[leucine aminopeptidase]], [[succinic dehydrogenase]], and [[phosphorylase]] and the immunohistochemical pattern of [[cytokeratin]] expression which indicates differentiation toward both the uppermost part of the dermal duct and the lower intraepidermal duct are characteristic findings of syringoma.
*On microscopic histopathological analysis, the histogenesis of syringomas is most likely related to eccrine or pluripotential stem cells.
*On microscopic histopathological analysis, the histogenesis of syringomas is most likely related to eccrine or pluripotential stem cells.
*On electron microscopy, ductal cells with numerous short microvilli, desmosomes, luminal tonofilaments, and lysosomes are characteristic findings of syringoma.
*On electron microscopy, ductal cells with numerous short microvilli, desmosomes, luminal tonofilaments, and lysosomes are characteristic findings of syringoma.

Revision as of 15:57, 12 August 2016

Syringoma
Syringomas highlighted by white circles.
ICD-10 D23.9
ICD-9 216
ICD-O: M8407/0
OMIM 186600
DiseasesDB 31358
MeSH D018252


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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Synonyms and keywords: Clear cell syringoma

Overview

Syringomas are eccrine sweat duct tumors, typically found clustered on eyelids, although they may also be found in the armpits, abdomen, chest, neck, scalp or groin area including genitals in a symmetric pattern. Syringomas are symmetrically distributed, usually multiple, and sometimes arranged in clusters. Syringoma is a benign adnexal neoplasm which is formed by well-differentiated ductal elements.[1]

Historical Perspective

  • The name syringoma is derived from the Greek word syrinx, which means pipe or tube.

Classification

  • Syringoma may be classified according to Friedman and Butler's classification into four subtypes:
  • A localized form
  • A form associated with Down syndrome
  • A generalized form that encompasses multiple and eruptive syringomas
  • A familial form

Location

The majority of syringomas are located on the upper parts of the cheeks and lower eyelids. Other common sites for syringomas include:

Syringomas limited to the dorsa of the hands have also been described. In the variant of eruptive syringoma, multiple lesions appear simultaneously on the chest and lower abdomen. However, rarely syringomas may appear as unilateral, linear, or nevoid lesions. The eruptive variant may involve the intertriginous areas and penis.[2][3][4]

Pathophysiology

Pathogenesis

The pathogenesis of syringoma is characterized by a hyperplastic response of the eccrine duct to an inflammatory reaction rather than a true adnexal neoplasm. Some authors have proposed the term "syringomatous dermatitis" for such cases. The scalp "syringomas" seen in scarring alopecia may represent a reactive proliferation in response to the fibrosis. It is hypothesized that phosphorylase deficiency, resulting from elevated glucose levels seen in diabetes leads to an accumulation of glycogen in the skin and within the clear cells.[5][6]*Syringoma is a benign neoplasm that differentiates along eccrine lines.

  • The pathophysiology of syringomas is largely unknown;however, the scientists propose that syringomas are benign growths that arise from the intraepidermal portion of eccrine ducts. It has also been suggested that a hamartoma of pluripotent stem cells could precede the pathological process of eruptive syringomas. Syringomas may also be under hormonal influence which may explain its female predominance.
  • The mutation in chromosome 16q22 has been associated with the development of familial autosomal dominant syringoma.

Associated Conditions

Microscopic Pathology

  • On immunohistochemical tests, the presence of eccrine enzymes such as leucine aminopeptidase, succinic dehydrogenase, and phosphorylase and the immunohistochemical pattern of cytokeratin expression which indicates differentiation toward both the uppermost part of the dermal duct and the lower intraepidermal duct are characteristic findings of syringoma.
  • On microscopic histopathological analysis, the histogenesis of syringomas is most likely related to eccrine or pluripotential stem cells.
  • On electron microscopy, ductal cells with numerous short microvilli, desmosomes, luminal tonofilaments, and lysosomes are characteristic findings of syringoma.

Causes

  • Syringoma may be caused by either sporadic mutation or in the setting of certain genetic disorders.
  • The mutation in chromosome 16q22 has been associated with the development of familial autosomal dominant syringoma..
  • Usually, syringomas are sporadic. Familial syringomas are usually inherited as an autosomal dominant trait, tend to occur in adolescence or preadolescence, and most commonly affect the face.[7][8][9][10][11]
  • If the syringoma is associated with Down syndrome, it is usually located in the periorbital region. However, some rare cases of eruptive syringomas associated with Down syndrome have also been reported. Clear-cell syringoma may be associated with diabetes mellitus.

Differentiating Syringoma from other Diseases

  • Syringoma must be differentiated from other diseases such as:

Epidemiology and Demographics

Syringomas affect approximately 1% of the population.

Age

  • Syringomas are more commonly observed at puberty. However, patients of all ages may be affected by syringomas.

Gender

  • Females are more commonly affected with syringoma than males.

Race

  • There is no racial predilection for syringoma.

Risk Factors

  • There are no established risk factors for syringoma.

Natural History, Complications and Prognosis

  • The majority of patients with syringomas remain asymptomatic and suffer no sequelae or complications.
  • Prognosis is generally excellent and with treatment, syringomas usually resolve with minimal scarring and no recurrence.

Diagnosis

History

  • A positive family history of syringoma may be present.

Symptoms

  • Syringomas are most commonly asymptomatic.
  • In some cases, patients with syringoma may present with pruritis.

Physical Examination

  • Physical examination may be remarkable for:
  • Skin-colored or yellowish, small dermal papules
  • Eruptive syringomas appear as hyperpigmented papules on the chest, penile shaft, or vulva
  • Lesions may appear translucent or cystic
  • Syringomas are commonly smaller than 3mm in diameter[12]

Laboratory Findings

  • There are no specific laboratory findings associated with syringoma.

Imaging Findings

  • There are no imaging findings associated with syringoma.

Other Diagnostic Studies

  • Syringoma may also be diagnosed using skin biopsy.
  • An adequate deep biopsy is usually required to rule out microcystic adnexal carcinoma.
  • Consideration of rebiopsy for persistent or recurrent lesions is indicated.

Treatment

Medical Therapy

  • The mainstay of medical therapy for syringoma is oral isotretinoin, topical atropine, topical tretinoin and oral tranilast.

Surgery

  • Surgery is the mainstay of therapy for syringomas.
  • The main reason for treatment is cosmetic; patients commonly seek treatment for syringomas of the cheeks and eyelids.
  • The goal of surgical therapy for syringomas should be the destruction of the tumor with minimal scarring and no recurrence. However, complete removal of syringomas is often unsuccessful and recurrence is common because syringomas are embedded within the dermis.
  • Possible treatments options available for syringomas include the following:
    • Surgical excision with primary suturing
    • Scissor excision with secondary intention healing
    • Electrocautery
    • Electrodesiccation and curettage
    • Carbon dioxide laser using the pinhole method of application or Er:YAG laser ablation
    • Fractionated carbon dioxide laser ablation
    • Cryotherapy
    • Dermabrasion
    • Trichloroacetic acid[13][14][15][16][17][18]

Prevention

  • There are no primary preventive measures available for syringoma. However, clinical follow-up with consideration of rebiopsy for persistent or recurrent lesions is indicated.

References

  1. ↑ syringoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Syringoma Accessed on April 26, 2016
  2. ↑ Koh MJ (2009). "Multiple acral syringomas involving the hands". Clin Exp Dermatol. 34 (7): e438. doi:10.1111/j.1365-2230.2009.03462.x. PMID 19663846.
  3. ↑ Petersson F, Mjörnberg PA, Kazakov DV, Bisceglia M (2009). "Eruptive syringoma of the penis. A report of 2 cases and a review of the literature". Am J Dermatopathol. 31 (5): 436–8. doi:10.1097/DAD.0b013e3181930d93. PMID 19542916.
  4. ↑ Sakiyama M, Maeda M, Fujimoto N, Satoh T (2014). "Eruptive syringoma localized in intertriginous areas". J Dtsch Dermatol Ges. 12 (1): 72–3. doi:10.1111/ddg.12203. PMID 24393317.
  5. ↑ Hashimoto K, Gross BG, Lever WF (1966). "Syringoma. Histochemical and electron microscopic studies". J Invest Dermatol. 46 (2): 150–66. PMID 4286059.
  6. ↑ Guitart J, Rosenbaum MM, Requena L (2003). "'Eruptive syringoma': a misnomer for a reactive eccrine gland ductal proliferation?". J Cutan Pathol. 30 (3): 202–5. PMID 12641781.
  7. ↑ Marzano AV, Fiorani R, Girgenti V, Crosti C, Alessi E (2009). "Familial syringoma: report of two cases with a published work review and the unique association with steatocystoma multiplex". J Dermatol. 36 (3): 154–8. doi:10.1111/j.1346-8138.2009.00613.x. PMID 19335690.
  8. ↑ Schepis C, Siragusa M, Palazzo R, Ragusa RM, Massi G, Fabrizi G (1994). "Palpebral syringomas and Down's syndrome". Dermatology. 189 (3): 248–50. PMID 7949476.
  9. ↑ Urban CD, Cannon JR, Cole RD (1981). "Eruptive syringomas in Down's syndrome". Arch Dermatol. 117 (6): 374–5. PMID 6454394.
  10. ↑ Ong GC, Lim KS, Chian LY (2010). "Eruptive syringoma in a patient with trisomy 21". Singapore Med J. 51 (2): e46–7. PMID 20358144.
  11. ↑ Jeon EK, Cho AY, Lee Y, Seo YJ, Park JK, Lee JH (2009). "Multiple yellow-brown papules on the neck and anterior trunk of a patient with diabetes mellitus". Clin Exp Dermatol. 34 (5): 643–4. doi:10.1111/j.1365-2230.2008.02847.x. PMID 19508481.
  12. ↑ Chan S, Millett C, Heymann WR (2012). "Giant syringomas: a case report". Cutis. 89 (1): 23–4. PMID 22439306.
  13. ↑ Jamalipour M, Heidarpour M, Rajabi P (2009). "Generalized eruptive syringomas". Indian J Dermatol. 54 (1): 65–7. doi:10.4103/0019-5154.48992. PMC 2800876. PMID 20049275.
  14. ↑ Al Aradi IK (2006). "Periorbital syringoma: a pilot study of the efficacy of low-voltage electrocoagulation". Dermatol Surg. 32 (10): 1244–50. doi:10.1111/j.1524-4725.2006.32284.x. PMID 17034373.
  15. ↑ Park HJ, Lee DY, Lee JH, Yang JM, Lee ES, Kim WS (2007). "The treatment of syringomas by CO(2) laser using a multiple-drilling method". Dermatol Surg. 33 (3): 310–3. doi:10.1111/j.1524-4725.2007.33065.x. PMID 17338688.
  16. ↑ Wang JI, Roenigk HH (1999). "Treatment of multiple facial syringomas with the carbon dioxide (CO2) laser". Dermatol Surg. 25 (2): 136–9. PMID 10037521.
  17. ↑ Cho SB, Lee SH, Lee SJ, Lee JE, Kim DH (2009). "Syringoma treated with pinhole method". J Eur Acad Dermatol Venereol. 23 (7): 852–3. doi:10.1111/j.1468-3083.2008.03051.x. PMID 19207646.
  18. ↑ Cho SB, Kim HJ, Noh S, Lee SJ, Kim YK, Lee JH (2011). "Treatment of syringoma using an ablative 10,600-nm carbon dioxide fractional laser: a prospective analysis of 35 patients". Dermatol Surg. 37 (4): 433–8. doi:10.1111/j.1524-4725.2011.01915.x. PMID 21414069.


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References

  1. ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 "Public Health Image Library (PHIL)".

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