Hailey–Hailey disease (also known as familial benign chronic pemphigus) is a blistering disease that can also include syringomas.Several systemic syndromes have also been associated with syringoma including diabetes mellitus, Down syndrome, Brooke–Spiegler syndrome, and Nicolau–Balus syndrome. Specifically, diabetes mellitus is strongly associated with clear cell syringoma consisting of nests of clear cells containing glycogen. A phosphorylase deficiency, resulting from elevated glucose levels seen in diabetes, is thought to lead to an accumulation of glycogen in the skin and within the clear cells. The incidence of syringomas has been reported in up to 40% of people with Down syndrome, and can be associated with a condition calcinosis cutis, which requires prompt medical attention. Brooke–Spiegler syndrome is a rare autosomal-dominant syndrome with cutaneous manifestations including syringomas and trichoepitheliomas. Nicolau–Balus syndrome is a rare autosomal-dominant disorder consisting of atrophoderma vermiculata and syringomas. Pathophysiology
The pathophysiology of syringomas remains largely unknown. Familial patterns presenting in an autosomal-dominant pattern suggest a genetic link that can result in varying genetic aberrations in lesions, specifically chromosome 16q22. The most commonly accepted theory is that syringomas are benign growths that arise from the intraepidermal portion of eccrine ducts. Another theory suggests that syringomas are a reactive hyperplasia rather than a true neoplasm resulting after inflammatory processes such as eczema. A hamartomatous process possibly could explain eruptive syringomas. A hamartoma of pluripotent stem cells could precede the pathological process. Syringomas may also be under hormonal influence, explaining the female predominance.
Diagnosis
Syringomas can often be diagnosed clinically based on presentation, distribution patterns over the body, lack of associated symptoms, and family history. A definitive diagnosis requires a skin biopsy to allow the tissue to be examined under a microscope. Histologically, syringomas have a characteristic comma-shaped ("tadpole") tail of dilated, cystic eccrine ducts. Treatment
The goal of treatment is to improve the appearance of lesions, since they are otherwise not serious and typically do not cause symptoms. Many treatment methods have been attempted, but complete removal is uncommon. No single treatment method has been shown to work consistently. Both medical and surgical treatments have been studied, each with variable success. Common destructive treatment methods include carbon dioxide lasers, dermabrasion, surgical excision, electrocoagulation, and chemical peels. Many of these methods are very time-consuming and require multiple treatment sessions. Carbon dioxide lasers are the most commonly practiced method; they can cause thermal damage, though, leading to scarring in the area. Medical therapies include topical atropine, topical retinoids, and oral tranilast. The most common adverse effects include redness, skin discoloration, and pain. Other side effects include blistering and scarring. See also
Acrospiroma
List of cutaneous conditions
List of cutaneous neoplasms associated with systemic syndromes

References
External links
eMedicine entry on syringomas
DermNet NZ