A connective tissue disorder characterized by widespread thickening of SKIN with a cobblestone-like appearance. It is caused by proliferation of FIBROBLASTS and deposition of MUCIN in the DERMIS in the absence of thyroid disease. Most scleromyxedema cases are associated with a MONOCLONAL GAMMOPATHY, immunoglobulin IgG-lambda.
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"Scleromyxedema is a rare dermatological disorder marked by widespread symmetric 2- to 3-mm, firm, waxy, closely spaced papules involving the hands, forearms, face, neck, upper trunk and thighs." | ( Fien, S; Jacob, SE; Kerdel, FA, 2006) |
"Scleromyxedema is an unusual skin disease characterized by mucinous infiltration of the skin." | ( Amini-Adle, M; Balme, B; Dalle, S; Thieulent, N; Thomas, L, 2007) |
"Scleromyxedema is notoriously difficult to treat." | ( Blanco, M; Efthimiou, P, 2008) |
"Scleromyxedema is a rare disorder characterized by mucin deposits in the dermis and monoclonal gammopathy." | ( Arpaci, F; Ataergin, S; Demiriz, M; Ozet, A, 2008) |
"Arndt-Gottron scleromyxedema is characterized by cutaneous mucinosis occasionally associated with systemic disorders, and it has a chronic and unpredictable course." | ( Guillet, G; Lopez, L; Villers, A; Wierzbicka-Hainaut, E, 2009) |
"Since Arndt-Gottron scleromyxedema is a rare disease, no standardized treatment has been yet established." | ( Guillet, G; Lopez, L; Villers, A; Wierzbicka-Hainaut, E, 2009) |
"Scleredema and scleromyxedema are attributed to increased hyaluronic acid, and lesional samples from these diseases also demonstrated accumulated dermal C4S." | ( Kim, JS; Werth, VP, 2011) |
"Scleromyxedema is associated with a monoclonal gammopathy and other comorbidities." | ( Calonje, E; Cinotti, E; Cozzani, E; Cribier, B; Fausti, V; Kanitakis, J; Kempf, W; Marinho, E; Merlo, G; Metze, D; Parodi, A; Rongioletti, F; Stefanato, CM, 2013) |
"The scleromyxedema is a rare condition characterized by hyperproliferation of fibroblasts with increased dermal deposition of mucin and frequently associated with monoclonal gammopathy of undetermined significance." | ( Caballero Escuti, G; Kurpis, M; Pascualini, MF; Ruiz Lascano, A; Valente, E, 2013) |
"Scleromyxedema is a rare generalized form of lichen myxedematosus, a chronic cutaneous mucinosis of unknown etiology usually associated with a monoclonal gammopathy and underlying systemic disorders." | ( Abarzúa, AA; Giesen, LF; González, SB; Sandoval, MO, 2014) |
"Scleromyxedema is a rare presentation for which a defined therapeutic regimen remains to be established." | ( Abarzúa, AA; Giesen, LF; González, SB; Sandoval, MO, 2014) |
"Scleromyxedema is a rare mucinous deposition disorder that shares clinical features with scleroderma but has important distinguishing features in clinical presentation and major organ complications that should be recognized." | ( Hummers, LK, 2014) |
"Scleromyxedema is a cutaneous mucinosis that is often accompanied by severe systemic manifestations." | ( Chockalingam, R; Duvic, M, 2016) |
"Scleromyxedema is a rare disorder that frequently affects multiple extracutaneous organ systems and is usually associated with monoclonal gammopathy." | ( Moritz, RKC; Neufeld, M; Sunderkötter, C, 2018) |
"Scleromyxedema is a rare cutaneous mucinosis with frequent extracutaneous manifestations." | ( Nair, M; Nair, SS; Poyuran, R; Vysakha, KV, 2019) |
"Scleromyxedema is a progressive, systemic connective tissue disorder characterized by fibro-mucous skin lesions and increased serum monoclonal immunoglobulin levels." | ( Al-Hilli, A; Kreidy, M; Resnick, J; Yachoui, R, 2020) |
"Scleromyxedema is a rare skin and systemic mucinosis that is usually associated with monoclonal gammopathy (MG)." | ( Arnulf, B; Aucouturier, P; Bagot, M; Barbarot, S; Battistella, M; Baubion, E; Beylot-Barry, M; Bouaziz, JD; Brault, F; Carpentier, O; Cribier, B; D'Incan, M; De Masson, A; de Moreuil, C; Debarbieux, S; Descamps, V; Dupuy, A; Fain, O; Frances, C; Hermine, O; Humbert, P; Jachiet, M; Le Bras, F; Le Moigne, M; Lifermann, F; Limal, N; Lipsker, D; Livideanu, CB; Mahévas, T; Mékinian, A; Michel, L; Modiano, P; Osio, A; Passeron, T; Peltier, S; Prud'homme, R; Rybojad, M; Sassolas, B; Sbidian, E; Schmutz, JL; Servy, A; Taha, RY; Talbot, A; Tauber, M; Thomas-Beaulieu, D; Zarnitsky, C, 2020) |
"Scleromyxedema is a chronic disease with high morbidity and mortality and no definitive therapeutic guidelines." | ( Bachour, J; El Gemayel, M; Haber, R, 2020) |
"Scleromyxedema is a rare primary cutaneous mucinosis characterized by numerous firm, waxy, confluent papules." | ( Akaslan, TÇ; Onsun, N; Yıldız, P, 2022) |
"Scleromyxedema is a rare idiopathic fibromucinous disorder characterized by a generalized papular and sclerodermoid cutaneous eruption." | ( Chen, QY; Wang, SS; Xiang, LH, 2022) |
Excerpt | Reference |
"Treatment of scleromyxedema is limited by the lack of long-term results, toxicity and significant adverse side effects." | ( Fien, S; Jacob, SE; Kerdel, FA, 2006) |
"Since Arndt-Gottron scleromyxedema is a rare disease, no standardized treatment has been yet established." | ( Guillet, G; Lopez, L; Villers, A; Wierzbicka-Hainaut, E, 2009) |
"We report an unusual case of scleromyxedema not associated with monoclonal gammopathy in a young patient who was treated with thalidomide." | ( Abarzúa, AA; Giesen, LF; González, SB; Sandoval, MO, 2014) |
"As the therapeutic approach to scleromyxedema remains challenging and treatment is based on symptomatic presentation, further clinical studies to substantiate an effective therapeutic regimen with a positive long-term safety and risk profile are required." | ( Abarzúa, AA; Giesen, LF; González, SB; Sandoval, MO, 2014) |
"We report a patient with scleromyxedema associated with hepatitis C virus, successfully treated with interferon and ribavirin therapy." | ( Erickson, CP; Kalimullah, FA; Peng, LS; Smith, JA, 2015) |
"An elderly patient with scleromyxedema developed pulmonary hypertension refractory to vasodilator and diuretic therapy and subsequently multiple myeloma that responded to a combination therapy of bortezomib, cyclophosphamide, and dexamethasone treatment." | ( Al-Hilli, A; Kreidy, M; Resnick, J; Yachoui, R, 2020) |