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Amyloidosis results from deposition of fibrous and insoluble amyloid protein in extracellular spaces of organs and tissues. Amyloid deposition can be localized or systemic and either primary or secondary. We report a case of localized primary cutaneous nodular amyloidosis manifested by papular-nodular, reddish-brown lesions affecting the nasal area, without evidence of systemic involvement. Immunohistochemistry showed the presence of immunoglobulin kappa light chain.

Brooke-Spiegler syndrome is an autosomal dominant inherited disease with predisposition to cutaneous adnexal neoplasms, most commonly cylindromas and trichoepitheliomas. Its onset is in the second or third decades of life. The histopathological exams of the lesions revealed a plethora of benign adnexal neoplasms, showing apocrine, follicular, and sebaceous differentiation. The treatment can be performed by excisional surgery, laser, cryotherapy, electrofulguration and dermabrasion. Due to the risk of malignancy, there is the need for clinical follow-up and genetic counseling.