Siringocistoadenoma Papilífero Congénito

Papillary syringocystoadenoma is an uncommon benign adnexal neoplasm, which is preferentially located on the scalp and face, and is associated with sebaceous nevus in 40% of cases. Although rare, its transition to basal cell carcinoma and ductal carcinoma may occur. The authors describe the case of a 7-year-old male patient with a linear papular dermatosis in the neck from birth. Dermatological examination showed erythematous, crateriform, confluent papules, arranged in a band, 3 x 2 cm in the cervical region. The lesion was excised for therapeutic and diagnostic purposes. Histopathology demonstrated epidermal cystic invagination and papillary projections surface by glandular epithelium, with decapitation secretion and abundant lymphoplasmocytic infiltrate. The clinical and histological findings were compatible with the diagnosis of papillary syringocystoadenoma. Despite the clinical variability of this entity, the histopathology is characteristic and the treatment consists in the excision of the lesion.