| Syringoma | |
|---|---|
 | |
| Syringomas highlighted by white circles | |
| Specialty | Oncology  |
Syringomas arebenigneccrine sweat ducttumors, typically found clustered oneyelids, although they may also be found in thearmpits,abdomen,chest,neck,scalp, orgroin area, includinggenitals, in a symmetric pattern.[1]: 663 They are skin-colored or yellowish firm, rounded bumps, 1–3 mm in diameter, and may be confused withxanthoma,milia,hidrocystoma,trichoepithelioma, andxanthelasma.[2] They are more common in women[3] and are most commonly found in middle-aged Asian women. While they can present at any time in life, they typically present duringadolescence. They are usually not associated with any othersymptoms, although can sometimes causeitchiness or irritation.[4]
Syringomas can be found in association with other symptoms as part of asyndrome.Hailey–Hailey disease (also known as familial benign chronic pemphigus) is ablistering disease that can also include syringomas.[5]
Several systemic syndromes have also been associated with syringoma includingdiabetes mellitus,Down syndrome,Brooke–Spiegler syndrome, andNicolau–Balus syndrome. Specifically, diabetes mellitus is strongly associated withclear cell syringoma consisting of nests of clear cells containingglycogen. Aphosphorylase deficiency, resulting fromelevated 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 conditioncalcinosis cutis, which requires prompt medical attention. Brooke–Spiegler syndrome is a rare autosomal-dominant syndrome with cutaneous manifestations including syringomas andtrichoepitheliomas. Nicolau–Balus syndrome is a rare autosomal-dominant disorder consisting ofatrophoderma vermiculata and syringomas.
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 reactivehyperplasia rather than a trueneoplasm resulting after inflammatory processes such aseczema. Ahamartomatous process possibly could explain eruptive syringomas. Ahamartoma ofpluripotent stem cells could precede the pathological process. Syringomas may also be under hormonal influence, explaining the female predominance.[citation needed]
Syringomas can often be diagnosed clinically based on presentation, distribution patterns over the body, lack of associated symptoms, andfamily history. A definitive diagnosis requires askin biopsy to allow the tissue to be examined under amicroscope. Histologically, syringomas have a characteristic comma-shaped ("tadpole") tail of dilated, cystic eccrine ducts.
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 includecarbon dioxide lasers,dermabrasion,surgical excision,electrocoagulation, andchemical 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 causethermal damage, though, leading toscarring in the area. Medical therapies includetopicalatropine, topicalretinoids, andoraltranilast.
The most commonadverse effects include redness, skin discoloration, and pain. Other side effects include blistering and scarring.