Answer: Answer to Dermacase

1. Syringoma

Syringoma presents with multiple small, firm, flesh- or yellow-coloured papules, occurs most often in women after puberty, and is possibly familial.1 The papules are benign adenomas of the eccrine ducts; although they most commonly appear on the eyelids, they can also be found on other areas of the face, the axillae, umbilicus, upper chest, and vulva. Histologically, the lesional pattern consists of many small ducts in the dermis with commalike tails, lending them a tadpolelike appearance.2 There are 4 clinical variants of syringoma: a localized form, a familial form, a form associated with Down syndrome, and a generalized form, which includes multiple and eruptive syringoma.3 Palpebral syringoma is a common cutaneous pathology in female adults with Down syndrome.4 It has been proposed that syringoma is partially under the hormonal influence of estrogen or progesterone, as it is more common in women and aggravated by pregnancy and menstruation. In a recent study of 61 patients with syringoma, however, the specimens were negative for estrogen and progesterone receptors; consequently, the pathophysiologic mechanism remains uncertain.5

Differential diagnoses

Xanthelasma presents on the eyelids and represents cholesterol deposition within dermal macrophages.1 In half of cases, it is associated with hyperlipidemia, which can be caused by a number of conditions: genetic disorders, diabetes mellitus, hypothyroidism, Cushing syndrome, pancreatitis, and renal disease. Hypercholesterolemia is associated with tendinous and planar xanthomas. Tuberous xanthomas are soft yellow-orange plaques or nodules usually found on extensor surfaces, particularly the elbows and knees. Patients with xanthomas should be investigated for hyperlipidemia.6

Trichoepithelioma is a benign appendageal tumour with differentiation toward hair. It typically tends to appear after puberty and can be inherited in an autosomal dominant pattern. Its lesions occur mostly on the face, and as they can look similar to those of syringoma a biopsy might be required to confirm diagnosis. One differentiating feature, however, is that trichoepithelioma does not frequently occur on the eyelids and often presents as solitary rather than multiple lesions. The lesions can grow quite large as well, unlike syringoma papules, which remain small.1

Cutaneous sarcoidosis can present with skin lesions around the eyes.1 The lesions also occur around the nose, on the back, and on the extremities. These are elevated lesions, are less than 1 cm in diameter, have a flat, waxy top, and can be brown to purple in colour.7

Sebaceous hyperplasia differs from syringoma in that the lesions usually scatter over the face rather than group in the periocular region. The lesions in sebaceous hyperplasia are often umbilicated.1 Sebaceous hyperplasia is more common in older patients and can be confused with small basal cell carcinoma.2

Treatment

Although they have no malignant potential, many patients with syringoma want the lesions treated for cosmetic reasons. This can be done by excision. The small mass is gently elevated, using either forceps or the curved bevel of a 25-gauge needle, then cut out with curved scissors or shaved with a scalpel blade. The oval wound is left to heal by secondary intention.8 Intralesional electrodesiccation is a safe and reliable method in trained hands.9 Laser ablation can also be very effective.10