Basal cell nevus syndrome (BCNS), or Gorlin Syndrome, is an autosomal dominant disorder, characterized by multiple developmental abnormalities and associated with germline mutations in the PTCH gene. Patients show multiple and early onset basal cell carcinomas (BCCs) in skin, odontogeniccysts in the jaw, pits on palms and soles, medulloblastoma, hypertelorism, and calcification of the falx cerebri. Clinical features of BCCs in these patients are indistinguishable from ordinary BCCs. However, some patients show variable histologic findings in subtypes of BCCs, and only one case associated with several histologic types of BCCs in the syndrome has been reported in Korea. We present a case of BCNS characterized by multiple BCCs, odontogenic keratocysts, multiple palmar pits, and calcified falx cerebri. Histopathologic findings of BCCs showed several patterns, which were nodular, superficial, and pigmented types.
Syringomas are common benign neoplasm of eccrine gland. Usually syringomas are encountered in face, especially periorbital region. However, in uncommon cases, these tumors arise at atypical site with discrete clinical feature. In other side, there has been lots of discussions that focus on relationship of alopecia, cicatrical or not, and syringoma. Herein, we report a case of syringoma as grouped yellowish papules in axillary region with localized alopecia.
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