Axillary syringomas mimicking Fox–Fordyce disease

A 25-year-old boy presented with 6 years history of asymptomatic papules over both axillae [Figure 1] and neck [Figure 2], and the development of similar papules around the eyes [Figure 3]. Physical examination revealed multiple, 1–2 mm in size, hyperpigmented, well-defined, oval to round papules over both axillae. There were similar papules on the flexor aspect of the neck, infraorbital area and trunk.

Figure 1.

Axillary papules

Figure 2.

Papules in beard region

Figure 3.

Infraorbital papules

Our differential diagnosis at this point of time was syringoma and Fox–Fordyce disease. A punch biopsy was performed. The histopathological findings of the papule on biopsy showed the dermis containing numerous ducts embedded in fibrous connective tissue stroma. The walls of the duct were lined by cuboidal to flattened epithelium, with a few containing eosinophilic material in their lumen. Some of the ducts had a comma shaped appearance [Figures 4 and 5]. These clinical findings were consistent with syringoma.

Figure 4.

H and E, ×10

Figure 5.

H and E, ×40

Syringoma is derived from the Greek word “syrnx,” which means pipe or tube. It is a benign adnexal tumor derived from epidermal eccrine ducts. Recent studies suggest that the lower acrosyringium or the transitional portion between the acrosyringium and the dermal duct as the point of origin.[1,2] The classification criteria of syringoma proposed by Friedman and Butler lists four variants:[3] Localized form, familial form, a form associated with Down's syndrome and a generalized form. Generalized syringoma has an earlier onset than localized syringoma. A rare variant, eruptive syringoma, described by Jacquet and Darier in 1987, occur in large numbers, in crops on the anterior chest, neck, upper abdomen, axillae, and the periumbilical region at puberty or during childhood among women. Eruptive syringoma is commonly seen in patients with Down's syndrome and Ehler–Danlos syndrome.[4]

Syringomas are usually located in the head and neck region, especially periorbitally.[5] They are less common on the scalp, forehead, cheeks, abdomen, extremities, axilla[6], genitalia and buttocks.[7] They clinically present as small, firm, skin-colored, yellow or brown papules.[8] Most studies, show a female to male ratio of 2:1, although some show a ratio as high as 6.6:1.[9,10] They occur mostly during puberty or during the third and fourth decades of life.[2]

Differential diagnoses for axillary syringoma are Darier's disease, Fox–Fordyce disease, Hailey–Hailey disease, plane warts, and keratosis pilaris.[11]

There is no permanent treatment for widespread syringomas, and surgical or chemical destruction involves some risk of scarring. Carbon dioxide lasers, topical tretinoin, trichloroacetic acid and dermabrasion have been found to be useful.[12]