Chronic Graft-versus-Host-Disease-Like Dermopathy in a Child with CD4+ Cell MicrochimerismKowalzick L.a · Artlett C.M.d · Thoss K.b · Baum H.-P.c · Ziegler H.a · Mischke D.a · Blum R.a · Pönnighaus J.-M.a · Quietzsch J.b
Departments of aDermatology and Allergology and bPediatric and Adolescence Medicine, Humaine Vogtland-Klinikum Plauen, Plauen, and cInstitute for Pathology, Zweibrücken, Germany; dDivision of Rheumatology, Thomas Jefferson University, Philadelphia, Pa., USA
Do you have an account?
- Rent for 48h to view
- Buy Cloud Access for unlimited viewing via different devices
- Synchronizing in the ReadCube Cloud
- Printing and saving restrictions apply
Rental: USD 8.50
Cloud: USD 20.00
We report the case of an 11-year-old boy suffering from a severe progressive chronic skin disease with clinical features of progressive systemic scleroderma, systemic lupus erythematosus and dermatomyositis. Skin biopsies revealed fibrosis and lichenoid changes and muscle biopsy a myositis. Immunohistology of the skin showed a lichen-ruber-like pattern. Despite repeated extensive investigations, no autoantibodies were detectable. Some of these findings looked like those described in juvenile dermatomyositis. Finally, it could be demonstrated that the boy showed microchimerism with approximately 1% maternal CD4+ lymphocytes in his peripheral blood leukocytes. Furthermore maternal cells could be demonstrated in inflamed muscle tissue. So a graft-versus-host-disease-like pathomechanism appears to be likely. Several systemic therapies have been used with limited success to improve the condition including corticosteroids, azathioprine, cyclosporine A and mycophenolate mofetil. A distinct improvement of erythemas and sclerosis could be achieved by means of low-dose UVA1 phototherapy which was applied with escalating single doses of 3–12 J/cm2 for 35 consecutive days.
© 2005 S. Karger AG, Basel
- Johnson ML, Farmer ER: Graft-versus-host reactions in dermatology. J Am Acad Dermatol 1998;38:369–392.
- Morita A, Kobayashi K, Isomura I, Tsuji T, Krutmann J: Ultraviolet A1 (340–400 nm) phototherapy for scleroderma in systemic sclerosis. J Am Acad Dermatol 2000;43:670–674.
von Kobyletzki G, Uhle A, Pieck C, Hoffmann K, Altmeyer P: Acrosclerosis in patients with systemic sclerosis respond to low-dose UVA1 phototherapy. Arch Dermatol 2000;44:548.
- Sönnichsen N, Meffert H, Kunzelmann V, Audring H: UVA-1 Therapie bei subakutem kutanem Lupus erythematodes. Hautarzt 1993;44:723–725.
- McGrath H: UV-A1 irradiation decreases clinical disease activity and autoantibodies in patients with systemic lupus erythematosus. Clin Exp Rheumatol 1994;12:129–135.
- MacGrath H, Martinez-Osuna P, Lee FA: Ultraviolet-A1 (340–400 nm) irradiation in systemic lupus erythematosous. Lupus 1996;5:269–274.
- Poldermann MC, Huizinga TW, LeCessie S, Pevel S: UV-A1 cold light treatment of SLE: A double blind, placebo controlled crossover trial. Ann Rheum Dis 2001;60:112–115.
- Grundmann-Kollmann M, Behrens S, Gruss C: Chronic graft-versus-host disease refractory to immunosuppressive treatment responds to UVA1 phototherapy. J Am Acad Dermatol 2000;42:134–136.
- Bianchi DW, Zickwolf GK, Weil GJ, Sylvester S, DeMaria MA: Male fetal progenitor cells persist in maternal blood for as long as 27 years postpartum. Proc Natl Acad Sci USA 1996;93:705–708.
- Nelson JL: Maternal-fetal immunology and autoimmune disease: Is some autoimmune disease allo-autoimmune or auto-alloimmune? Arthritis Rheum 1996;39:191–194.
- Artlett CM, Smith JB, Jimenez SA: Identification of fetal DNA and cells in skin lesions from women with systemic sclerosis. N Engl J Med 1998;338:1186–1191.
- Miyashita Y, Ono M, Ono M, Ueki H, Kurasawa K: Y chromosome microchimerism in rheumatic autoimmune disease. Ann Rheum Dis 2000;59:665–666.
- Artlett CM, Cox LA, Jimenez SA: Detection of cellular microchimerism of male or female origin in systemic sclerosis patients by polymerase chain reaction analysis of HLA-Cw antigens. Arthritis Rheum 2000;43:1062–1067.
- Artlett CM, Ramos R, Jiminez SA, Patterson K, Miller FW, Rider LG: Chimeric cells of maternal origin in juvenile idiopathic inflammatory myopathies. Lancet 2000;356:2155–2156.
- Reed AM, Picornell YJ, Harwood A, Kredich DW: Chimerism in children with juvenile dermatomyositis. Lancet 2000;356:2156–2157.
- Eppinger T, Ehninger G, Steinerz M, Niethammer D, Dopfer R: 8-Methoxypsoralen and ultraviolet A therapy for cutaneous manifestations of graft-versus-host disease. Transplantation 1990;50:807–811.
- Kapoor N, Pelligrini AE, Copelan EA, Cunningham I, Avalos BR, Klein JL: Psoralen plus ultraviolet A (PUVA) in the treatment of chronic graft-versus-host disease: Preliminary experience in standard treatment resistant patients. Semin Hematol 1992;29:108–112.
Dall’Amico R, Zulian F, Montini G, Andretta B, Murer L, Rosetti F, Livi U, Zacchello Q, Zacchello F: Applications of extracorporeal photochemotherapy in ‘non-oncological’ diseases. Int J Artif Organs 1993;16(suppl 5):168–172.
- Owsianowski M, Gollnik H, Siegert W, Schwerdtfeger R, Orfanos CE: Successful treatment of chronic graft-versus-host disease with extracorporeal photopheresis. Bone Marrow Transplant 1994;14:845–848.
- Aractingi S, Khosrotehrani K: Microchimerism: Fears and hopes. Dermatology 2005;210:1–2.
Article / Publication Details
Copyright / Drug Dosage / DisclaimerCopyright: 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.