Review
Evaluation and management of the patient with multiple syringomas: A systematic review of the literature

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Syringomas are benign adnexal tumors with distinct histopathologic features, including the characteristic comma (“tadpole”) shaped tail comprised of dilated, cystic eccrine ducts. Clinically, syringomas typically present in adolescent females predominantly in the periorbital region. They may present as solitary or multiple lesions, and more rare sites of involvement include the genitals, palms, scalp, and the chest. Over the past 50 years, there have been >800 reported cases of syringoma either alone or in conjunction with a systemic syndrome, most commonly Down syndrome. The primary aim of this systematic review is to discuss the clinical features and associations of syringomas with a focus on the patient with multiple syringomas. Its secondary aims are to explore pathophysiology with a focus on multiple syringomas and provide comprehensive data on both traditional and novel treatments. Importantly, multiple syringomas present across a broad clinical spectrum. Though noted in many textbooks to be related to tumor syndromes, the association of syringomas with inherited tumor syndromes is only rarely reported in the literature. Despite multiple reported cases of syringoma, the pathophysiology remains poorly understood and treatment continues to pose a significant challenge.

Section snippets

Search strategies

A systematic literature search was conducted on the PubMed and Scopus databases using the following search terms: “familial syringoma,” “hereditary syringoma,” “familial eruptive syringoma,” “hereditary eruptive syringoma,” “syringoma,” “familial generalized syringoma,” “generalized syringoma,” “multiple syringoma,” “eruptive syringoma,” “vulvar syringoma,” “periorbital syringoma,” “penile syringoma,” “scalp syringoma,” “axilla syringoma,” “milia AND syringoma,” “lichen AND syringoma,” “plaque

Results

Eight hundred twenty-six cases of syringomas were reported in the literature between 1964 and 2013. Comprehensive data were obtained from 239 cases of syringoma identified from 46 reports2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 4 that met the inclusion criteria. Two hundred fifteen cases described treatment,17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32,

Clinical presentation, prevalence, and systemic associations

This systematic review highlights important patterns that may be relevant in the assessment of a patient presenting with multiple syringomas, including unusual presentations, familial cases, and rare associated syndromes. The common occurrence of syringomas on the genitalia, scalp, and axilla and eruptive cases with lesions distributed over the trunk and extremities necessitate a total body skin examination. The anatomic distribution in familial cases and nonfamilial cases varied slightly, with

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    Funding sources: None.

    Conflicts of interest: None declared.

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