A 53-year-old female presented with itchiness in her back. She has a 5 years history of tightness of the skin on her face, neck, and torso bilaterally. She did not have other symptoms suggestive of systemic sclerosis (scleroderma), and her rheumatologic workup was negative. Skin biopsy showed increased dermal mucin confirming a diagnosis of scleredema. Further workup with serum protein electrophoresis (SPEP) showed an M-spike, confirming the diagnosis of monoclonal gammopathy of undetermined significance MGUS as the underlying pathology.

serum protein electrophoresis (SPEP), monoclonal gammopathy of undetermined significance (MGUS), double-stranded DNA (dsDNA), antibodies immunoglobulin G (ANA IgG), anti-Sjogren’s syndrome (SSB), antitopoisomerase antibody-type (SCL), ribonucleic acid (RNA), cyclic citrullinated peptide (CCP), U1 small nuclear ribonucleoprotein particle (U1 RNP), multiple myeloma (MM), immunomodulatory drugs (IMiDs), proteasome inhibitors (PIs), intravenous immunoglobulins (IVIG)

[1] Ioannidou, D. I., Krasagakis, K., Stefanidou, M. P., Karampekios, S., Panayiotidis, J., & Tosca, A. D. (2005). Scleredema adultorum of Buschke presenting as periorbital edema: a diagnostic challenge. Journal of the American Academy of Dermatology, 52(2 Suppl 1), 41–44.

[2] Jablonska, S., & Blaszczyk, M. (1998). Scleroderma-like disorders [Internet]. Seminars in Cutaneous Medicine and Surgery, 17, 65–76.

[3] Beers, W. H., Ince, A., & Moore, T. L. (2006). Scleredema adultorum of Buschke: A case report and review of the literature. Seminars in Arthritis and Rheumatism, 35(6), 355–359.

[4] Alp, H., Orbak, Z., & Aktas, A. (2003). Scleredema adultorum due to streptococcal infection. Pediatrics International, 45(1), 101–103.

[5] Angeli-Besson, C., Koeppel, M. C., Jacquet, P., Andrac, L., Sayag, J. (1994). Electron-beam therapy in scleredema adultorum with associated monoclonal hypergammaglobulinaemia. British Journal of Dermatology, 130(3), 394–397.

[6] Kyle, R. A., Durie, B. G. M., Rajkumar, S. V., Landgren, O., Blade, J., Merlini, G., Kröger, N., Einsele, H., Vesole, D. H., Dimopoulos, M., San Miguel, J., Avet-Loiseau, H., Hajek, R., Chen, W. M., Anderson, K. C., Ludwig, H., Sonneveld, P., Pavlovsky, S., Palumbo, A., … Boccadoro, M. (2010). Monoclonal gammopathy of undetermined significance (MGUS) and smoldering (asymptomatic) multiple myeloma: IMWG consensus perspectives risk factors for progression and guidelines for monitoring and management. Leukemia, 24(6), 1121–1127.

[7] Mitchell, D. C., Agnihothri, R., Scott, G. A., Korman, B., & Richardson, C. T. (2019). A case of concurrent systemic sclerosis and scleredema. JAAD Case Reports, 5(11), 940–942.

[8] Orteu, C. H., Ong, V. H., & Denton, C. P. (2020). Scleroderma mimics - Clinical features and management. Best Practice & Research: Clinical Rheumatology, 34(1), 101489.

[9] McFadden, N., Ree, K., Søyland, E., & Larsen, T. E. (1987). Scleredema adultorum associated with a monoclonal gammopathy and generalized hyperpigmentation. Archives of Dermatology, 123(5), 629–632.

[10] Sala, A. C. B., Cunha, P. R., Pinto, C. A. L., Alves, de Moraes Alves, C. A. X., Paiva, I. B., & Araujo, A. P. V. (2016). Scleromyxedema: Clinical diagnosis and autopsy findings. Anais Brasileiros de Dermatologia, 91(5 suppl 1), 48–50.

[11] Popović, D., Paravina, M., Jovanović, D., Karanikolić, V., & Ljubisavljević, D. (2016). Scleromyxedema (Arndt-Gottron Syndrome): A case report. Serbian Journal of Dermatology and Venereology, 8, 28–38.

[12] Rongioletti, F., & Rebora, A. (2001). Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema. Journal of the American Academy of Dermatology, 44(2), 273–281.

[13] Basarab, T., Burrows, N. P., Munn, S. E., & Russell Jones, R. (1997). Systemic involvement in scleredema of Buschke associated with IgG-kappa paraproteinaemia. British Journal of Dermatology, 136(6), 939–942.

[14] Chang, H. K., Kim, Y. C., & Kwon, B. S. (2004). Widespread scleredema accompanied with a monoclonal gammopathy in a patient with advanced ankylosing spondylitis. Journal of Korean Medical Science, 19, 481.

[15] Ho, M., Patel, A., Goh, C. Y., Moscvin, M., Zhang, L., & Bianchi, G. (2020). Changing paradigms in diagnosis and treatment of monoclonal gammopathy of undetermined significance (MGUS) and smoldering multiple myeloma (SMM). Leukemia, 34(12), 3111–3125.

[16] Barnes, M., Kumar, V., Le, T.-H., Nabeel, S., Singh, J., Rana, V., Kaell, A., & Barnes, H. (2020). A case of paraproteinemia-associated scleredema successfully treated with thalidomide. JAAD Case Reports, 6(10), 1039–1041.

[17] Rongioletti, F., Kaiser, F., Cinotti, E., Metze, D., Battistella, M., Calzavara-Pinton, P. G., Damevska, K., Girolomoni, G., André, J, Perrot, J.-L., Kempf, W., Cavelier-Balloy, B. (2015). Scleredema. A multicentre study of characteristics, comorbidities, course and therapy in 44 patients. Journal of the European Academy of Dermatology and Venereology, 29(12), 2399–2404.

[18] Rongioletti, F., & Rebora, A. (2001). Cutaneous mucinoses: microscopic criteria for diagnosis. American Journal of Dermatopathology, 23(3), 257–267.

[19] Könemann, S., Hesselmann, S., Bölling, T., Grabbe, S., Schuck, A., Moustakis, C., De Simoni, D., Willich, N., & Micke, O. (2004). Radiotherapy of benign diseasesscleredema adultorum Buschke. Strahlentherapie und Onkologie, 180(12), 811–814.

[20] Cole, G. W., Headley, J., & Skowsky, R. (1983). Scleredema diabeticorum: A common and distinct cutaneous manifestation of diabetes mellitus. Diabetes Care, 6(2), 189– 192.

[21] Venencie, P. Y., Powell, F. C., Su, W. P., & Perry, H. O. (1984). Scleredema: A review of thirty-three cases. Journal of the American Academy of Dermatology, 11(1), 128–134.

[22] Keragala, B. S. D. P., Herath, H. M. M. T. B., Janappriya, G. H. D. C., Dissanayaka, B. S., Shyamini, S. C., Liyanagama, D. P., Balendran, T., Constantine, S. R., & Gunasekera, C. N. (2019). Scleredema associated with immunoglobulin A-κ smoldering myeloma: A case report and review of the literature. Journal of Medical Case Reports, 13(1), 145.

[23] Tran, K., Boyd, K. P., Robinson, M. R., & Whitlow, M. (2013). Scleredema diabeticorum. Dermatology Online Journal, 19(12), 20718.