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[Regional mucocutaneous plasmacytosis]
E Bartolo1, P Cachäo, L Garcia e Silva
1Serviço de Dermatologia e Venerologia, Hospital de Santa Maria, Lisboa, Portugal.
Abstract:
The case of a 48 year old woman is reported with confluent nodules on the base of her nose and adjacent infraorbital areas, isolated ones on her face, and endotracheal masses with severe lumen reduction. Histologically the dermis and chorion of the nasal and tracheal mucosa were infiltrated by normal looking plasma cells. There was an increased ESR and a polyclonal gammapathy but no radiological evidence of skeletal involvement, bone marrow infiltration by atypical plasma cells or Bence Jones proteinuria. Cobalt-therapy was rapidly effective in reducing the endotracheal masses. The skin lesions were unaffected by roentgen-therapy and by several chemotherapeutic schedules. Laboratory values almost reverted to normal and her general condition has been satisfactory for the last two years. The differential diagnosis with multiple myeloma and extramedullary plasmocytoma was discussed and the condition classified as chronic, probably benign, mucocutaneous plasma cell infiltration.