Abstract
A case of idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia characterized by cutaneous involvement was reported.
A 40-year-old woman was admitted because of skin rashes, generalized lymphadenopathy and fever. Since about 20 years of age the skin rashes which were brownish pigmentation and erythematous macules with slight purpura have always appeared over the trunk and the extrimities. In the bilateral axillary and inguinal nodes there were enlarged lymph nodes, 2×3 cm in maximum diameter. Hepatosplenomegaly was present.
Hypochromic microcytic anemia with polyclonal hyperimmunoglobulinemia was observed. Serum Fe, TIBC, transferrin were low and ferritin was elevated. Bone marrow aspiration revealed 4.2% plasma cells without atypsim. STS, PPD, DNCB and various autoantibodies were not disclosed. The right inguinal lymph node biopsy revealed follicular hyperplasia with germinal centers, prominent mature plasma cell proliferation between follicles and dilatation of the sinus. Histological examination of the skin rashes showed moderate infiltration of mature plasma cells and slight hemorrhage in perivascular area of the dermis.
Lymphadenopathy, skin rashes, fever and hypergammaglobulinemia were improved gradually by COP (cyclophosphamide, vincristine, prednisolone) therapy.
