A 71-year-old man was referred to our department due to a painful nodule on the left temporal area. The tumor had been increasing in size for 7 years since its onset. Physical examination revealed a dome-shaped reddish nodule measuring 1.5 cm in diameter on the left aspect of the scalp. There was also a smaller reddish nodule with a peculiar appearance in the same region. Histopathologically, the specimen from the larger nodule showed a nodular lesion composed of multiple basophilic nests surrounded by basaloid cells and with an eosinophilic hyaline sheath between the nests in the whole dermis, forming a so-called jigsaw puzzle structure. Based on these characteristic findings, a diagnosis of cylindroma was established. The smaller reddish nodule showed pathological findings compatible with senile angioma, but the clinical manifestation was not typical in view of its size. Although he had no familial history of specific skin tumors including cylindroma and adnexal skin tumors, the possibility of Brooke-Spiegler syndrome (BSS) was considered because of his past history of parotid gland tumor. In the Japanese literatures, there have been reports of some sporadic cases of BSS who had cylindroma accompanied with other adnexal tumors, parotid grand tumor or lung cancer. These observations may suggest that sporadic cases of cylindroma might have a localized or systemic mutation in the CYLD gene.
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