Syringoma Localized to the Umbilicus

Dear Editor:

Syringomas are relatively common and benign appendageal tumors derived from the eccrine glands. Syringomas typically appear on the eyelid, but rarely can arise on the skin in other areas1. Although eruptive syringomas may also involve the periumbilical area, it is very rare for the lesions to be only limited to this region. Here, we report a rare case of a patient with periumbilical syringomas.

A 25-year-old male presented with numerous quiescent, localized papules in his periumbilical area, with a history of several years. On physical examination, there were multiple 1~2 mm sized, firm, light-brown colored papules in the periumbilical area (Fig. 1A). A closer examination with a dermoscope revealed multiple pink to whitish areas surrounded by finely pigmented network (Fig. 1B). The histopathological examination revealed multiple ductal and small cystic structures, dispersed within a fibrous connective tissue stroma in the upper dermis. The ductal and cystic spaces were lined by 1 to 2 rows of flattened epithelial cells (Fig. 1C). The patient was diagnosed with localized periumbilical syringomas, however he refused further management.

Fig. 1
(A) Multiple, small, slightly raised, brown colored papules located in the periumbilical area. (B) Multiple whitish to pink areas and fine pigmented network were observed under the dermoscope. (C) The histopathological examination showed multiple ductal and small cystic structures embedded within a fibrous connective tissue stroma. The ducts were lined by 1 to 2 rows of epithelial cells. Some ducts showed a “tadpole”-like appearance (H&E, ×100). We received the patient's consent form about publishing all photographic materials.

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A syringoma is an organoid tumor with an intraepidermal eccrine ductal differentiation2. Typical lesions are multiple, flesh colored to yellow-brown dermal papules that may be soft or cystic in appearance and are movable over the subcutaneous tissue. Syringomas occur about twice as frequently in females and may either be localized or generalized in distribution3. The skin of the eyelids and the malar regions are the most common sites of involvement, although syringomas may also appear on the neck, anterior chest, axillae, arms, and abdomen3, 4, 5. However, syringomas localized solely to the periumbilical area have never been reported in literature.

The diagnosis is uncomplicated in syringomas involving a preferred site such as the lower eyelids. However, syringomas occurring in unusual locations are likely to be misdiagnosed, as e.g. Fox-Fordyce disease. But the lesions in Fox-Fordyce disease are smaller, more conical, and periodically very pruritic. Epidermal cysts or milia also could be confused with syringomas. They are usually fewer in number, more whitish and yellowish in appearance, and there are often one or more lesions with a characteristic central punctum in epidermal cysts5. Flat warts also occur as multiple, yellow-brown, small papules, but they have a hyperkeratotic flat-topped surface with confluent features and gradually spread by scratching or trauma. Clinically, it may be possible to distinguish, but biopsy is an essential diagnostic tool to prevent unnecessary treatments that waste the patient's time and resources.

Dermoscopic findings of periorbital syringoma show ivory to white colored homogenous areas with poorly defined borders, which were also observed in our case. The difference is that the hyperpigmented network is more prominent in our case because of the structural characteristics of the umbilicus.

Our report is of interest due to the unusual appearance of syringomas only involving the periumbilical area. We suggest that syringomas should be considered during the diagnosis and management of patients presenting only with papular lesions of the periumbilical area. To our knowledge, this is the first such reported case.