Cutaneous involvement in prelymphomatous angioimmunoblastic lymphadenopathy

• Published in: Journal of the American Academy of Dermatology 1997-02, Vol.36 (2), p.290-295
• Main Authors: Schmuth, Matthias, Ramaker, Jutta, Trautmann, Christoph, Hummel, Michael, Schmitt-Gräff, Anette, Stein, Harald, Goerdt, Sergij
• Format: Article
• Language: English
• Subjects: Aged; Biological and medical sciences; Dermatitis - etiology; Diagnosis, Differential; Drug Eruptions - diagnosis; Female; Hematologic and hematopoietic diseases; Humans; Immunoblastic Lymphadenopathy - complications; Immunoblastic Lymphadenopathy - diagnosis; Medical sciences; Other diseases. Hematologic involvement in other diseases
• ISSN: 0190-9622; 1097-6787
• DOI: 10.1016/S0190-9622(97)80401-X

We describe prelymphomatous angioimmunoblastic lymphadenopathy with cutaneous involvement in a 73-year-old female patient. A maculopapular skin eruption was the first sign of the disease. Skin histology showed extensive perivascular and periadnexal mixed lymphoid infiltrates including centroblasts and immunoblasts with a high proliferative index and with focal erythrocyte extravasation. Lymph node histology confirmed the diagnosis, showing nearly complete effacement of the follicular architecture, a mixed lymphoid infiltrate, and numerous high endothelial venules in an expanded T-cell zone. Immunohistochemistry, however, demonstrated preservation of at least some follicular structures. T-cell receptor gene rearrangement analysis revealed oligoclonal patterns in both lymph node and skin specimens. In contrast, immunoglobulin heavy-chain gene rearrangement analysis revealed a polyclonal pattern. Accordingly, the disease was classified as a prelymphomatous stage of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) with specific involvement of both lymph node and skin. The patient was treated with high-dose corticosteroids, and long-lasting remission was induced. In contrast to our case, most reported cases of AILD show a monoclonal T-cell pattern indicating AILD-type lymphoma. Therefore we discuss the concept of prelymphomatous AILD developing into AILD-type lymphoma. Persistence of some antigenic stimulus may induce the proliferation of a monoclonal population of lymphoid cells from a polyclonal background in a multistep fashion. Proper treatment of AILD at an early, prelymphomatous stage may protract or inhibit development of full-blown, fatal AILD-type lymphoma.