Hereditary syningomas, a case report
Published Web Locationhttps://doi.org/10.5070/D33cm9x77g
Hereditary syningomas: A case report
Department of Dermatology, University of California Davis
Michelle Draznin MD
Dermatology Online Journal 10 (2): 19
Syringoma is a benign neoplasm of eccrine origin. Clinically, it is an eruption of small translucent-to-yellowish papules. These lesions are firm, smooth, and approximately 1-3 mm in diameter. They are most commonly found around the eyes and on the upper cheeks of middle-aged women. Lesions sometimes develop on the abdomen, axillae, penis, vulva, and scalp. Involvement of the scalp may be indistinguishable from nonscarring alopecia. Familial cases have been reported, and there is an increased incidence of syringoma in adults with Down syndrome. Eruptive syringoma, a separate entity, presents mostly in adolescents as clusters of numerous papules on the upper half of the body.
A 54-year-old man presented to the Veterans Affairs Medical Center for a skin check. He had seen a dermatologist in the distant past for lesions around his eyes. The patient stated that a biopsy was performed at that time, and it revealed that these lesions were "salt crystals" in the skin. He is not bothered by these lesions and is not interested in any further interventions since the previous dermatologist told him that their removal would result in significant scarring.
|Figure 1||Figure 2|
The patient is a healthy-appearing man who has skin findings limited to the periorbital area. He has bilateral periorbital edema with slightly yellowish papules surrounding the eyes. The papules are nontender and without central changes. The papules appear to be concentrated along the lower eyelid. No similar lesions are present elsewhere, including on the cheeks and forehead.
Following significant search efforts, we were unable to obtain the patient's biopsy results so, with the patient's consent, we performed another biopsy. We elected to do a shave biopsy since the patient desired minimal "fuss" near his eye.
|Figure 3||Figure 4|
Histopathology reveals aggregations of dilated cystic sweat ducts in the dermis. Most are characterized by comma-like tails giving them a tadpole shape. The biopsy specimen demonstrates nests of epithelial cells in the dermis. Often, these epithelial cells are surrounded by collagen bundles. We did not perform the histochemical studies to demonstrate hydrolytic enzymes typical of eccrine origin; the clinical and histologic evidendce was adequate to support the diagnosis of syringoma.
Syringoma is a benign eccrine sweat gland tumor. It is categorized by location: eyelid, scalp, genital, and acral region. Tumors appear as single or as clusters of small papules and may be skin colored or slightly pigmented. They are equally common pre- and post-puberty. Prepubertal eruptions have a predilection for apocrine sites such as the axillae and pubis. These sites are rarely involved in postpubertal subjects . Most studies that have reviewed patient presentations note that the clinical diagnosis of syringoma, particularly eruptive syringoma, was usually not suspected . The definitive diagnosis of syringoma was made by histologic exam. Typically, a haematoxylin-eosin stain shows multiple, small, dilated ducts and epithelial cords in the dermis. Most of these ducts have comma-like tails associated with the surrounding epithelial cells.
Recent literature has challenged the notion that eruptive syringoma is neoplastic. These authors maintain that eruptive syringoma actually represents a hyperplastic response of the eccrine duct to an inflammatory reaction [3, 4]. They present two specific case reports of syringoma occurring in response to another process. The first case outlines a 41-year-old man with a diffuse erythematous macular and papular eruption preceding the development of skin-colored papules, proven to be syringomas by histopathology. The second case describes a 27-year-old male who developed an eczematous eruption on his wrist where he had been wearing a watch. After the eruption had faded, he developed tan papules outlining the watch site. These were also found to be syringomas by histopathology .
Most authors still believe syringoma to be of sporadic or hereditary etiology. Our patient informed us that his father had similar lesions around his eyes. He also said that his sister has similar but fewer lesions. Familial syringoma is rare, but possibly underestimated in prevalence. This form appears to be autosomal dominant . Interestingly, syringoma appears to be more common in patients with Marfan, Down, and Ehlers-Danlos syndromes . There is no one single genodermatosis with a phenotype signigicant only for syringoma.
Treatment of syringoma is often frustrating. Most of the literature suggests using carbon dioxide laser [5, 6]. Other treatment modalities include excision, electrodessication and curettage, and dermabrasion. One study demonstrates good results with temporary tattooing following Q-switched alexandrite laser . Unfortunately, all surgical interventions result in scarring. Our 54-year-old veteran did not ask for treatment and is just delighted that these lesions are benign.
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