European Journal of Dermatology


Brooke‐Spiegler syndrome with parotid gland involvement Volume 14, numéro 3, May - June 2004

Department of Surgery, Thraki Medical Center, 1 Komninon Str, 68100 Alex\polis, Greece Alex\polis Radiodiagnostic Center, 59 Venizelou Str, 68100 Alex\polis, Greece Department of Pathology, Theagenio Anticancer Hospital, 2 Simeonidi Str, Thessalonica, Greece

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.