Scleromyxedema and Monoclonal Gammopathy
https://doi.org/10.35754/0234-5730-2026-71-1-108-117
Abstract
Introduction. Monoclonal gammapathy is a heterogeneous group of diseases associated with the formation of a clone of plasma cells in the bone marrow and the secretion of paraprotein.
Aim: to present clinical observations demonstrating the effectiveness of therapy in patients with scleromyxedema associated with monoclonal gammapathy.
Main findings. Scleromyxedema is one of the dermatological manifestations of monoclonal gammapathy. It is characterized by thickening of the skin due to excessive formation and deposition of mucin in the dermis, involvement of internal organs in the process. Without therapy, this condition can cause complications from the gastrointestinal tract, respiratory, cardiovascular and nervous systems. An effective treatment method is clone-reducing therapy using proteasome inhibitors, antitumor immunomodulators, alkylating drugs, glucocorticoids, as well as subsequent melphalan chemotherapy, and autologous hematopoietic table cell transplantation. As a result of the treatment, regression of dermatological manifestations and complete reduction of the clone of plasma cells were achieved.
About the Authors
A. E. Grachev
National Medical Research Center for Hematology
Russian Federation
Russian Federation
Alexander E. Grachev, Cand. Sci. (Med.), Hematologist of the Department of Chemotherapy for Hematological Diseases
125167, Moscow
E. A. Shatokhina
National Medical Research Center for Hematology ; Central State Medical Academy under the Administrative Department of the President of the Russian Federation
Russian Federation
Russian Federation
Evgeniya A. Shatokhina, Dr. Sci. (Med.), Professor, Dermatovenerologist
125167, Moscow
121359, Moscow
A. D. Valitova
National Medical Research Center for Hematology
Russian Federation
Russian Federation
Azaliya D. Valitova, Hematologist
125167, Moscow
D. B. Churkina
Central State Medical Academy under the Administrative Department of the President of the Russian Federation
Russian Federation
Russian Federation
Darya B. Churkina, Dermatovenerologist
121359, Moscow
A. A. Shumilova
Research Institute of Rheumatology named after V.A. Nasonova
Russian Federation
Russian Federation
Anastasiia A. Shumilova, Junior Researcher, Rheumatologist
115522, Moscow
E. O. Gribanova
National Medical Research Center for Hematology
Russian Federation
Russian Federation
Elena O. Gribanova, Cand Sci. (Med), Head of the Department of Chemotherapy for Hematological Diseases
125167, Moscow
References
1. Kyle R.A., Larson D.R., Therneau T.M., et al. Long-term follow-up of monoclonal gammopathies of undetermined signifi cance (MGUS). N Engl J Med. 2018;59(6):639–40. DOI: 10.1007/s00108-018-0425-2.
2. Fermand J.P., Bridoux F., Dispenzieri A., et al. Monoclonal gammopathy of clinical signifi cance: A novel concept with therapeutic implications. Blood. 2018;132(14):1478–85. DOI: 10.1182/blood-2018-04-839480.
3. Claveau J-S., Wetter D.A., Kumar S. Cutaneous manifestations of monoclonal gammopathy. Blood Cancer J. 2022;12(4):1–14. DOI: 10.1038/s41408-022-00661-1.
4. Ríos-Tamayo R., Paiva B., Lahuerta J.J., et al. Monoclonal Gammopathies of Clinical Signifi cance: A Critical Appraisal. Cancers. 2022;14(21):5247. DOI: 10.3390/cancers14215247.
5. Karakus S., Gottsch J.D., Caturegli P. E.A. Monoclonal gammopathy of «ocular” signifi cance. Am J Ophthalmol Case Reports. 2019;15:100471. DOI: 10.1016/j.ajoc.2019.100471.
6. Kanack A.J., Schaefer J.K., Sridharan M., et al. Monoclonal gammopathy of thrombotic/thrombocytopenic signifi cance. Blood. 2023;141(14):1772–6. DOI: 10.1182/blood.2022018797.
7. Rongioletti F., Merlo G., Cinotti E., et al. Scleromyxedema: a multicenter study of characteristics, comorbidities, course, and therapy in 30 patients. J Am Acad Dermatol. 2013;69(1):66–72. DOI: 10.1016/j.jaad.2013.01.007.
8. Hoffmann J.H.O., Enk A.H. Scleromyxedema. J Dtsch Dermatol Ges. 2020;18(12):1449–67. DOI: 10.1111/ddg.14319.
9. Dinneen A.M., Dicken C.H. Scleromyxedema. J Am Acad Dermatol. 1995;33(1):37–43. DOI: 10.1016/0190-9622(95)90007-1.
10. Cokonis Georgakis C.D., Falasca G., Georgakis A., et al. Scleromyxedema. Clin Dermatol. 2006;24(6):493–7. DOI: 10.1016/j.clindermatol.2006.07.011.
11. Harper R.A., Risper J. Lichen myxedematosus serum stimulates human skin fi broblast proliferation. Science. 1978;199:545–7. DOI: 10.1126/science.622555.
12. Radenska-Lopovok S.G., Volkova P. Is scleromyxedema a skin problem or systemic pathological process? Arkh Patol. 2018;80(1):63–6. DOI: 10.17116/patol201880163-66.
13. Lafyatis R. Transforming growth factor β- At the centre of systemic sclerosis. Nat Rev Rheumatol. 2014;10(12):706–19. DOI: 10.1038/nrrheum.2014.137.
14. Rothhammer T., Braig S., Bosserhoff A.K. Bone morphogenetic proteins induce expression of metalloproteinases in melanoma cells and fi broblasts. Eur J Cancer. 2008;44(16):2526–34. DOI: 10.1016/j.ejca.2008.07.029.
15. Bos R., de Waal E.G.M., Kuiper H., et al. Thalidomide and dexamethasone followed by autologous stem cell transplantation for scleromyxoedema. Rheumatology. 2011;50(10):1925–6. DOI: 10.1093/rheumatology/ker209.
16. Mahévas T., Arnulf B., Bouaziz J.D., et al. Plasma cell — directed therapies in monoclonal gammopathy — associated scleromyxedema. Blood. 2020;135(14):1101–10. DOI: 10.1182/blood.2019002300.
17. Theves F., Lahuna C., Mahévas T., et al. Plasma cell-directed therapies induce profound clinical and durable responses in patients with severe or relapsed/refractory scleromyxedema. J Eur Acad Dermatology Venereol. 2025;39(5):1011– 6. DOI: 10.1111/jdv.20257.
18. Lvov A.N., Sheklakova M.N., Matushevskaya Y.I. Arndt — Gottron scleromyxedema. Vestn Dermatol Venerol. 2012; DOI: 10.25208/vdv733.
19. Karim M.M.B. Case 5: extensive skin eruption causing skin to become tight, sore, and itchy. Diagnosis: scleromyxoedema. Clin Exp Dermatol. 2003;28(3):343–4. DOI: 10.1017/cbo9780511750878.026.
20. Espinosa A., De Miguel E., Morales C., Fonseca E.G.-B.J. Scleromyxedema associated with arthritis and myopathy: a case report. Clin Exp Rheumatol. 1993;11(5):545–7.
21. Jamieson T.W., De Smet A.A., Stechschulte D.J. Erosive arthropathy associated with scleromyxedema. Skeletal Radiol. 1985;14(4):286–90. DOI: 10.1007/BF00352621.
22. De Simone C., Castriota M., Carbone A., et al Cardiomyopathy in scleromyxedema: report of a fatal case. Eur J Dermatol. 2010;20(6):852–3. DOI: 10.1684/ejd.2010.1099.
23. Nieves D.S., Bondi E.E., Wallmark J., et al. Scleromyxedema: successful treatment of cutaneous and neurologic symptoms. 2000; 65(2):89–92.
24. Berger J.R., Dobbs M.R., Terhune M.H., Maragos W.F. The neurologic complications of scleromyxedema. Medicine. 2001;80(5):313–9. DOI: 10.1097/00005053-194405000-00004.
Review
For citations:
Grachev A.E., Shatokhina E.A., Valitova A.D., Churkina D.B., Shumilova A.A., Gribanova E.O. Scleromyxedema and Monoclonal Gammopathy. Russian journal of hematology and transfusiology. 2026;71(1):108-117. (In Russ.) https://doi.org/10.35754/0234-5730-2026-71-1-108-117
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