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Syringomas

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Common benign sweat gland tumors that originate from the straight portion of the intradermal eccrine sweat duct.

Present as skin colored to slightly yellowish papules commonly found in the periorbital area.

Usually appears at puberty or in the third and fourth decades of life.

Refer to harmless eccrine sweat duct tumors.

They are 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.

They appear as skin-colored or yellowish firm, rounded bumps, 1–3 mm in diameter, and may be confused with xanthoma, milia, hidrocystoma, trichoepithelioma, and xanthelasma.

More common in women and are most commonly found in middle-aged Asian women.

Can present at any time in life.

Typically present during adolescence.

Not usually associated with any other symptoms although can sometimes cause itchiness or irritation.

An eruptive form typically presents on the anterior chest, abdomen, neck and arms.

Eruptive form presents in successive crops with periods of relief in between times of active rash.

Milia-like form of syringoma is typically smaller lesions that have a milky white center that can look like milia.

Plaque type is more commonly associated with itchiness and chronic scratching that leads to epidermal thickening.

Some cases exhibit a familial pattern in an autosomal dominant pattern of inheritance.

Chromosome 16q22 has been shown to be involved in the genetic links of syringoma.

Hailey-Hailey disease a blistering disease that can also include syringomas.

Systemic processes associated with syringoma including diabetes mellitus, and down syndrome.

Diabetes mellitus is strongly associated with clear cell syringoma consisting of nests of clear cells containing glycogen.

The incidence of syringomas has been reported in up to 40 percent of people with Down syndrome.

Can be associated with a calcinosis cutis which requires prompt medical attention.

Familial patterns presenting in an autosomal dominant pattern.

Genetic aberrations of chromosome 16q22.

Theory is that they are benign growths that arise from the intraepidermal portion of eccrine ducts, or are a reactive hyperplasia rather than a true neoplasm resulting after inflammatory processes such as eczema.

A hamartomatous process could explain eruptive syringomas, and may also be under hormonal influence explaining the female predominance.

Clinically based diagnosis: distribution patterns over the body, lack of associated symptoms and family history.

A definitive diagnosis requires a skin biopsy, as syringomas have a characteristic comma shaped tail of dilated, cystic eccrine ducts.

Treatment attempts to improve the appearance of lesions.

No single treatment method has been shown to consistently effective.

Treatment methods include: carbon dioxide lasers, dermabrasion, surgical excision, electrocoagulation and chemical peels.

Medical therapies include topical atropine, topical retinoids and oral tranilast.

Prevalence is 0.6% of the population and is higher Asian and African Americans.

Female to male ratio is approximately 2:1 in most studies.

Most cases are sporadic, familial cases have been reported.

Familial cases are likely to be autosomal dominant in inheritance.

Process is more common in Down syndrome, Ehlers-Danlos syndrome, Castillo syndrome, Marfan syndrome, Nicolau-Balus syndrome, and diabetes mellitus.

Pathogenesis is unknown but is hypothesized to represent a hyperplastic response of the eccrine sweat duct by keratin plugs, leading to ductsl proliferation may play a role.

Hormonal factors may be involved since the process is more common in women and there is a peak incidence during puberty.

Histologically manifest for a small dilated eccrine ducts within the dense fibrous stroma.

Eccrine ducts are lined by 2 rows of cuboidal to flattened epithelial cells, the outer laying layer bulging outwards and giving rise to the characteristic tadpole appearance.

Ductal lumina are filled with an amorphous periodic acid-Schiff positive material.

Patients present with small soft to firm, skin colored to slightly yellow papules that are usually 1-3 mm in diameter, asymptomatic and symmetrically distributed.

While the lesions may be solitary, they are most often multiple in nature.

The lesions may be localized or generalized.

Localized lesions are the most common clinical variant, and are usually found in the periorbital area.

Generalized lesions are found mainly on the chest or neck, followed by the forearms.

Lesions may appear on other body areas such as the penis, axilla, and buttocks.

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