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 Printable version |
Brooke‐Spiegler syndrome with parotid gland involvement |
European Journal of Dermatology. Volume 14, Number 3, 139-41, May - June 2004, Genes and skin
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 Free Article
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Author(s) : Despoina KAKAGIA, George ALEXIADIS, Anastasia KIZIRIDOU, Maria LAMBROPOULOU |
Summary : Salivary gland involvement in Brooke‐Spiegler syndrome (BSS), an autosomal dominantly inherited disease, is known though not frequent. A case of familial cylindromatosis with parotid gland adenoma is herein reported. A 67‐year‐old lady presented with multiple scalp nodules and papular coalescent lesions over the nasolabial folds and the forehead. The clinical examination also revealed a left preauricular lump. Multiple biopsies of the scalp lesions and the nasolabial papules revealed cylindromas and trichoepitheliomas respectively. CT scan and FNA of the preauricular lump were suggestive of parotid gland adenoma. The patient underwent excision of the scalp cylindromas and total left parotidectomy. There is no evidence of recurrence after 4 years. The association of BSS with salivary gland tumours, emphasizes the necessity of thorough salivary gland examination in all patients with skin lesions. Knowledge of the genetic background of BSS allows for genetic counseling of patients. |
Keywords : familial cylindromatosis, inherited appendageal tumors, parotid gland adenoma, turban tumor |
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