Excision and split thickness skin grafting in the treatment of nodular primary localized cutaneous amyloidosis.

摘要

Nodular primary localized cutaneous amyloidosis (NPLCA) is the rarest of the cutaneous amyloidoses. The amyloid is derived from immunoglobulin light chains (AL protein) produced by local plasma cells . Histological findings are indistinguishable from those of primary systemic amyloidosis, so additional clinical and laboratory evaluation must be performed, and careful follow up is recommended. There is no consistent treatment for NPLCA, particularly when large areas are involved, and local recurrence rate is high .A 68-year-old Caucasian woman was referred for possible surgical treatment to our department in 1996. For the preceding three years she had had skin nodules on the anterior tibial region of her right leg, gradually increasing in size and number. The area of the skin involved measured 23 x 15 centimetres. The nodules were painful and frequently ulcerated and infected, causing the patient distress. Physicalexamination revealed five firm, non fixed, ovoid, shiny, brown-pink nodules on the anterior tibial region of right leg, which ranged in size up to 5 cm in diameter and which were surrounded by healthy skin. The overlying skin on three of the nodules was ulcerated.