Syringoma

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.

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
• Familial form

Pathophysiology

Location

The majority of syringomas are located on the upper parts of the cheeks and lower eyelids. The other characteristic sites for syringomas include the following:

• The axilla
• Chest
• Abdomen
• Penis
• Vulva.

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.

Associated Conditions

• Syringomas can be found in association with other symptoms as part of a syndrome such as Hailey-Hailey disease also known as familial benign chronic pemphigus, diabetes mellitus, Down syndrome, Brooke-Spiegler, and Nicolau-Balus. Diabetes mellitus is strongly associated with clear cell syringoma consisting of nests of clear cells containing glycogen. 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.
• 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.
• 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.

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.

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.
• 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:

• Acne Vulgaris
• Apocrine Hydrocystoma
• Basal Cell Carcinoma
• Colloid Milium
• Cowden Disease(Multiple Hamartoma Syndrome)
• Favre-Racouchot Syndrome(Nodular Elastosis With Cysts and Comedones)
• Granuloma Annulare
• Microcystic Adnexal Carcinoma
• Milia
• Sebaceous Hyperplasia
• Steatocystoma Multiplex
• Trichoepithelioma
• Tuberculosis
• Xanthelasma

Epidemiology and Demographics

Syringomas affect approximately 1% of the population. Syringomas are benign lesions and are largely of cosmetic significance.

Age

• Patients of all age groups may develop syringoma. However, Syringomas are more commonly observed at puberty.
• Syringomas are most commonly found in middle-aged Asian women.

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.
• Prognosis is generally excellent and with treatment, syringomas ideally should be destroyed with minimal scarring and no recurrence.

Diagnosis

History

• A positive family history of syringoma may be present.
• The majority of patients with syringoma are asymptomatic.

Symptoms

• Syringoma is usually asymptomatic.
• Symptoms of syringoma may include the following:

• 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
• Usually, syringomas are smaller than 3mm in diameter

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

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.

Gallery

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  Syringoma From Public Health Image Library (PHIL).

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  Syringoma From Public Health Image Library (PHIL).

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  Syringoma From Public Health Image Library (PHIL).

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  Syringoma From Public Health Image Library (PHIL).

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  Syringoma From Public Health Image Library (PHIL).

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  Syringoma From Public Health Image Library (PHIL).

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  Syringoma From Public Health Image Library (PHIL).