Familial syringomas.

Sir,

A 60-year-old female patient presented with multiple, flat topped and few dome-shaped papules mainly confined to the eyelids and cheeks with comparatively fewer lesions on the forehead and chin. The lesions started in the second decade of life and increased in number over a period of 10 years and were asymptomatic [Figure 1]. No similar history was present in her parents and siblings. Two daughters were affected with the same disease. Systemic and cutaneous examination did not reveal anything else significant. Biopsy was done from the lesion on the chin and histopathology revealed epithelial neoplasm involving the upper reticular dermis made up of solid epithelial islands and ductal structures. The ductal structures were lined by two or three layers of cuboidal cells. The same cells present within solid cords showed attempt towards ductal differentiation. The stroma was made up of thickened bundles of collagen. The findings were consistent with syringomas [Figures 2 and 3]. The elder daughter was 38 year old at the time of presentation and had shiny, papules, size ranging from 2 to 5 mm mainly on the forehead, eyelids, cheeks, and chin with few lesions scattered on the remaining areas of face. The lesions appeared at the age of 34 years and were progressively increasing in number [Figure 4]. The younger daughter, 35 year old presented with shiny, dome-shaped papules mainly on the eyelids and cheeks which started at the age of 20 years.

Figure 1

Clinical photograph showing multiple syringomas on the face of the affected mother in the family

Figure 2

Histopathology showing an epithelial neoplasm involving the upper reticular dermis made up of solid epithelial islands and ductal structures lined by two or three layers of cuboidal cells (H and E, ×100)

Figure 3

Histopathology showing an epithelial neoplasm involving the upper reticular dermis made up of solid epithelial islands and ductal structures lined by two or three layers of cuboidal cells (H and E, ×400)

Figure 4

Clinical photograph showing multiple syringomas in one of the daughters affected with the disease

There are only a few reports of the familial occurrence of syringomas. A major textbook of dermatology, “Textbook of Dermatology”, states that syringomas do not appear to be hereditary.[1] Syringoma is a benign appendageal tumor of eccrine acrosyringeal differentiation which usually manifests as multiple, flesh-colored papules around the eyelids and upper cheeks of adolescent females.[2] Friedman and Butler proposed a classification, based on the clinical features and associations. This consisted of four principal clinical variants of syringoma: A localized form, a familial form, a form associated with Down's syndrome, and a generalized form that encompasses multiple and eruptive syringoma.[3]

Three patients with a positive family history of syringoma have been reported and all were females.[4] In most reported families, syringomas have been observed to occur in an autosomal dominant pattern and the condition tends to be more common in females. This female preponderance in both sporadic and familial forms of syringoma is well-recognized, but remains unexplained.[4] There is another interesting report of familial syringoma in three generations with two cases associated with Steatocystoma multiplex.[5] Familial occurrence of the tumor may not be as rare as generally believed.[6]