We describe a case of systemic sclerosis (SSc) occurring together with malignant lymphoma. A 43-year-old man, who had noticed sclerodactyly 1 month before consultation, was admitted for progressive skin sclerosis on his forearms and chest. SSc was diagnosed. Immediately after admission, skin sclerosis rapidly extended to the neck and trunk, and subcutaneous tumors developed on the neck, chest and back. Skin sclerosis was prominent at the sites where subcutaneous tumors were present. The tumors were diagnosed as non-Hodgkin’s lymphoma of T-cell phenotype derived from soft tissue. Following 4 cycles of chemotherapy, he had complete remission and the skin sclerosis remarkably improved. It is possible that cytokines produced by T-cell lymphoma cells were responsible for the development of skin sclerosis in this case.

Keywords:
Systemic sclerosis, Malignant lymphoma, Non-Hodgkin’s lymphoma
1.
Willemze R, Kerl H, Sterry W, Cerroni S, Chimenti S, Diaz-Perez JL, Geerts ML, Goos M, Knobler R, Ralfkiaer E, Santucci M, Smith N, Wechsler J, van Vloten WA, Meijer CJLM: EORTC classification for primary cutaneous lymphomas: A proposal from the cutaneous lymphoma study group of the European Organization for Research and Treatment of Cancer. Blood 1997;90:354–371.
2.
Joost TV, Stolz E, Blog FB, Van der Kwast TH, Vuzevski VD: B-cell markers in malignant B-cell lymphoma with scleroderma-like manifestation. J Cutan Pathol 1984;11:495–500.
3.
Keder A, Khan AB, Mattern JQA, Fisher J, Thomas PRM, Freeman AI: Autoimmune disorders complicating adolescent Hodgkin’s disease. Cancer 1979;44:112–116.
4.
Hall SW, Gillespie JJ, Tenczynski TF: Generalized lipodystrophy, scleroderma, and Hodgkin’s disease. Arch Intern Med 1978;138:1303.
5.
Katz AL, Nashel DJ, Goard CP, Bauer H: Pemphigus vulgaris and lymphoma in a patient with scleroderma. South Med J 1979;72:1463– 1467.
6.
Sugai S, Takiguchi T, Hirose Y, Shimizu S, Tachibana J, Konda S, Konishi F: B cell malignant lymphoma in a patient with progressive systemic sclerosis and Sjögren syndrome. Jpn J Med 1985;24:155–163.
7.
Doyle JA, Connolly SM, Hoagland HC: Hematologic disease in scleroderma syndromes. Acta Derm Venereol (Stockh) 1985;65:521– 525.
8.
Sugai S, Tachibana J, Sawada M, Shimizu S, Hirose Y, Takiguchi T, Konda S: Malignant lymphomas in patients with autoimmune diseases: a report of 6 cases and a review of Japanese literature. Jpn J Med 1987;26:339–347.
9.
Lens XM, Fernandez E, Panades MJ, Casanova JM, Macia JM, Ramos J, Montolin J: Progressive systemic sclerosis and palmar hyperkeratosis as a paraneoplastic syndrome in non-Hodgkin’s lymphoma. Nephrol Dial Transplant 1992;7:1166–1167.
10.
Comer M, Harvey AR: Remission of scleroderma during chemotherapy for lymphoma. Ann Rheum Dis 1992;51:998–1000.
11.
Yamamoto T, Furuse Y, Nishioka K: Progressive systemic sclerosis preceded by malignant lymphoma. J Dermatol 1993;20:648–650.
12.
Baldini L, Guffanti A, Ferrari A, Castagnone D, Mascagni B, Colombi M, Maiolo AT: Uncommon clinical presentation of a lymphocytic lymphoma of intermediate differentiation in a patient with systemic sclerosis. Br J Haematol 1994;86:657–658.
13.
Suzuki A, Ohoike H, Kitagawa Y, Matsuoka Y, Irimajiri S: Progressive systemic sclerosis complicated with primary cerebral malignant lymphoma. Inter Med 1994;33:557–559.
14.
Parma M, Sita G, Guffanti A, Bianchi F, Colombi M, Baldini L: Multilobated B cell lymphoma (Pinkus variant) with isolated muscular localization in a patient with systemic sclerosis: A case report. Ann Hematol 1996; 73:43–45.
15.
Kassan SS, Thomas TL, Moutsopoulos HM, Hoover R, Kimberly RP, Budman DR, Costa J, Decker JL, Chused TM: Increased risk of lymphoma in sicca syndrome. Ann Intern Med 1978;89:888–892.
16.
LeRoy EC, Smith EA, Kahaleh MB, Trojanowska M, Silver RM: A strategy for determining the pathogenesis of systemic sclerosis: Is transforming growth factor β the answer? Arthritis Rheum 1989;32:817–825.
17.
Higley H, Persichitte K, Chu S, Waegell W, Vancheeswaran R, Black C: Immunocytochemical localization and serologic detection of transforming growth factor β1: Association with type I procollagen and inflammatory cell markers in diffuse and limited systemic sclerosis, morphea, and Raynaud’s phenomenon. Arthritis Rheum 1994;37:278–288.
1999
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.