Basal cell nevus syndrome

Basal cell nevus syndrome or xeroderma pigmentosum The efficacy and safety of imiquimod cream have not been established for patients with basal cell nevus syndrome or xeroderma pigmentosum. 

R. Rifkin, B. Reed, F. Hetzel, K. Chen (1997). Photodynamic therapy using SnET2 for basal cell nevus syndrome a case report. Clin. Then, 19, 639-641. 

Nodular, morphea-like and superficial BCC were examined however, CR was not given for the different histologic types. 26-year-old man with basal cell nevus syndrome. 

Finally, recent research has indicated that other human diseases, such as the commonest form of skin cancer, basal cell carcinoma, are caused by mutations in genes involved in the Sonic hedgehog signalling pathway. The Patched (Ptc) gene encodes a Sonic hedgehog receptor [92,93] and a tumor suppressor protein that is defective in basal cell nevus syndrome [94]. 

Fibromas are composed mostly of fibroblasts and spindle cells and abundant collagen contents. Fibromas are not hormonally active. Fifteen percent of fibromas are associated with ascites (Fig. 9.32), and in 1% pleural fluid is also found [83]. This triad of an ovarian fibroma, ascites and pleural effusion constitutes the benign Meigs syndrome, which can be associated with elevated Ca-125 levels [84]. In basal cell nevus syndrome, numerous basal cell carcinomas are associated with abnormalities of bones, eyes, brain, and tumors, including ovarian bilateral fibromas [85]. 

A successful prevention of basal cell carcinomas and squamous cell carcinomas in patients with Xeroderma pigmentosum has been reported for systemic isotretinoin [46] and etretinate therapy [3]. Over a period of two years, isotretinoin was shown to reduce the occurrence of basal cell carcinomas by 80% and of squamous cell carcinomas by 60%. For etretinate, a reduction of 75% was reported with respect to the prevention of squamous cell carcinomas. Both retinoids have also been shown to be effective in preventing basal cell carcinomas in organ transplant recipients [40] and in the basal cell nevus syndrome [28, 34]. Furthermore, a combination of topical tretinoin and low-dose oral etretinate (10 mg/d) has been suggested for chemoprevention with reduced side-effects [69]. 

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