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

Historical Perspective

[Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].

Classification

Syringoma may be classified according to 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

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, [cause2], or [cause3].
[Disease name] is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
There are no established causes for [disease name].

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.[3]

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

The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

Patients of all age groups may develop [disease name].
[Disease name] is more commonly observed among patients aged [age range] years old.
[Disease name] is more commonly observed among [elderly patients/young patients/children].

Gender

[Disease name] affects men and women equally.
[Gender 1] are more commonly affected with [disease name] than [gender 2].
The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

There is no racial predilection for [disease name].
[Disease name] usually affects individuals of the [race 1] race.
[Race 2] individuals are less likely to develop [disease name].

Risk Factors

Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

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

Diagnostic Criteria

The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:

[criterion 1]
[criterion 2]
[criterion 3]
[criterion 4]

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

Patients with [disease name] usually appear [general appearance].
Physical examination may be remarkable for:

[finding 1]
[finding 2]
[finding 3]
[finding 4]
[finding 5]
[finding 6]

Laboratory Findings

There are no specific laboratory findings associated with [disease name].

A [positive/negative] [test name] is diagnostic of [disease name].
An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings

There are no [imaging study] findings associated with [disease name].
[Imaging study 1] is the imaging modality of choice for [disease name].
On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

[Disease name] may also be diagnosed using [diagnostic study name].
Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

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].