Syringoma

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

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2] Synonyms and keywords: Synonym 1; Synonym 2; Synonym 3

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.

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.^[1]^[2]^[3]

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.^[4]^[5]^[6]^[7]^[8]
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

Syringomas are more commonly observed at puberty. However, additional lesions can be observed later.

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 [disease name] remain asymptomatic for [duration/years].
Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
Prognosis is generally [excellent/good/poor], and the [1/5/10¬year mortality/survival rate] of patients with [disease name] is approximately [#%].

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^[9]

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

There is no treatment for [disease name]; the mainstay of therapy is supportive care.
The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
[Medical therapy 1] acts by [mechanism of action1].
Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

Surgery is the mainstay of therapy for [disease name].
[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
[Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

There are no primary preventive measures available for [disease name].
Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].