An unusual presentation of eruptive syringomas on the neck

Abstract

Dermatologic manifestations can be the initial signs of a systemic disease, and a thorough investigation may be warranted to rule out a life-altering diagnosis. We present a case of an 11-year-old boy with a concurrent presentation of eruptive syringomas and juxtaclavicular beaded lines on his neck. While a biopsy confirmed the benign diagnosis, the initial presentation led to a concerning differential including pseudoxanthoma elasticum and warranted a complete investigation.

Eruptive syringomas and juxtaclavicular beaded lines are two separate entities that typically present in varying demographics. We present a case of concurrent eruptive syringomas and juxtaclavicular beaded lines in an 11-year-old boy, which resulted in a worrisome initial differential including pseudoxanthoma elasticum (PXE).

CASE REPORT

A healthy 11-year-old boy presented with a 5-year history of asymptomatic persistent papules on his neck. The papules became more prominent with heat and exercise but were neither pruritic nor bothersome. The patient’s medical history included seasonal allergies and myopia requiring glasses. He was recently examined by his ophthalmologist with no abnormal findings. The remainder of the skin was uninvolved. There were scattered 2 to 5 mm yellow-orange papules on a background of smaller, countless skin-colored papules arranged horizontally in a linear pattern, on the anterior neck and extending bilaterally (Figure 1a). A 3 mm punch biopsy of a larger papule on the anterolateral neck revealed comma-shaped epithelial structures consistent with syringoma, so a diagnosis of eruptive syringomas was made (Figure 1b). The patient’s clinical presentation warranted a concurrent diagnosis of juxtaclavicular beaded lines.

Figure 1.

(a) View of the anterior neck showing scattered 2 to 5 mm yellow-orange papules on a background of smaller, countless skin-colored papules arranged horizontally in a linear pattern. (b) Skin biopsy showing comma-shaped epithelial structures (arrowheads) in the dermis (hematoxylin and eosin, original magnification 20×).

DISCUSSION

Given the location of the lesions on the anterolateral neck, the age of the patient, and coalescing appearance of the papules, concern for pseudoxanthoma elasticum (PXE) was raised. PXE is a rare, autosomal recessive disease caused by the loss of a protein that regulates mineralization of connective tissue.¹ Calcification of elastic fibers results in multiorgan manifestations, most commonly involving the skin, arteries, and retina.¹ The first presenting sign is usually an area of yellow-tan papules or plaques located on flexural areas, most commonly the lateral neck.¹ The papules on our patient looked to be considerably denser in some areas, resembling the plaque-like formation of PXE; however, there have been reports of eruptive syringomas progressing into plaques.²

Syringomas are benign adnexal tumors derived from the intraepidermal portion of eccrine sweat ducts and present as soft, flesh-colored to slightly yellow dermal papules.³ They have a predilection for the lower eyelids, cheeks, axillae, neck, and abdomen and predominantly occur in women at puberty and in patients with Down syndrome.³ Although patients with syringomas present at a mean age of 29 years,³ studies examining patients with eruptive syringomas show presentation between 4 and 10 years of age, similar to our patient.⁴

Adding to the diagnostic conundrum was the concurrent presence of juxtaclavicular beaded lines. This is a benign anatomical variant of sebaceous hyperplasia presenting as parallel lines of papules resembling strings of pearls usually localized in juxtaclavicular areas, but also commonly found at the nape of the neck, inguinal region, and axillae.⁵ While this entity is commonly reported in dark-skinned adults, there are minimal reports of fair-skinned and prepubescent children presenting with this variant of sebaceous hyperplasia. The etiology is unknown; however, hormonal influences, sun exposure, and links to corticosteroid treatment have been noted.⁵

Our patient’s dual presentation of eruptive syringomas on a background of juxtaclavicular beaded lines contributed to our concern for more serious pathology such as pseudoxanthoma elasticum. The patient and his family were reassured of the benign nature, and no treatment was deemed necessary.