Scleromyxedema (Lichen Myxedematosus)

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.

Also Known As:

Networked: 258 relevant articles (33 outcomes, 9 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Paraproteinemias (Monoclonal Gammopathy)
2.	Mucinoses
3.	Multiple Myeloma
4.	Hypothyroidism
5.	Lymphoproliferative Disorders (Lymphoproliferative Disorder)

Experts

1.	Rongioletti, F: 4 articles (05/2020 - 10/2000)
2.	Rongioletti, Franco: 4 articles (03/2018 - 03/2008)
3.	Cribier, Bernard: 3 articles (01/2020 - 07/2013)
4.	Hummers, Laura K: 3 articles (01/2020 - 01/2008)
5.	Kempf, Werner: 3 articles (06/2016 - 01/2004)
6.	Marinho, Eduardo: 3 articles (06/2016 - 07/2013)
7.	Rebora, A: 3 articles (03/2001 - 10/2000)
8.	Aucouturier, Pierre: 2 articles (03/2021 - 01/2020)
9.	Bouaziz, Jean-David: 2 articles (03/2021 - 01/2020)
10.	Carpentier, Olivier: 2 articles (03/2021 - 01/2020)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Scleromyxedema:

1.	Intravenous Immunoglobulins (IVIG)FDA Link 06/01/2007 - "[Marked improvement in scleromyxedema with high-dose intravenous immunoglobulin]." 06/01/2007 - "High-dose intravenous immunoglobulin appears to be a promising treatment option for patients with scleromyxedema. " 05/01/2022 - "More studies are required to investigate the potential efficacy of IVIG in the treatment of scleromyxedema." 06/01/2009 - "In conclusion, increasing evidence supports intravenous immunoglobulin as an effective and relatively safe treatment for both cutaneous and extracutaneous manifestations of scleromyxedema, including the dermatoneuro syndrome." 07/01/2020 - "Long-Term Efficacy of Treatment with Intravenous Immunoglobulin in Scleromyxedema."
2.	Thalidomide (Thalomid)FDA Link 01/01/2006 - "We report a severe case of scleromyxedema who had marked improvement when treated with thalidomide." 05/01/2013 - "Successful treatment of scleromyxedema with oral thalidomide." 01/01/2013 - "[Response to thalidomide in scleromyxedema]." 12/01/2008 - "Intravenous gammaglobulin and thalidomide may be an effective therapeutic combination in refractory scleromyxedema: case report and discussion of the literature." 01/01/2007 - "Scleromyxedema: successful treatment with thalidomide in two patients."
3.	Melphalan (Alkeran)FDA LinkGeneric 08/01/2008 - "Complete and durable remission in a patient with life-threatening scleromyxedema treated with high-dose melphalan and BU with auto-SCT." 10/01/2016 - "In an original study published in 2006, seven patients with scleromyxedema were treated with high-dose melphalan and autologous PBSCT between April 2000 and November 2003. " 05/01/2017 - "Atypical Lichen Myxedematosus: A Case with Remarkable Response to Low Dose Melphalan." 11/01/1984 - "[Lichen myxedematosus, I. Statistical studies on associated liver injury and successful treatment with melphalan]." 03/01/1979 - "Treatment of scleromyxedema with melphalan."
4.	Dexamethasone (Maxidex)FDA LinkGeneric 10/01/2005 - "High-dose dexamethasone in scleromyxedema: report of 2 additional cases." 08/01/2004 - "A complete and durable clinical response to high-dose dexamethasone in a patient with scleromyxedema." 01/01/2017 - "A case of scleromyxedema responding to lenalidomide and dexamethasone." 01/01/2005 - "Successful treatment of scleromyxedema with dexamethasone cyclophosphamide pulse therapy." 10/01/2011 - "Scleromyxedema and dermato–neuro syndrome in a patient with multiple myeloma effectively treated with dexamethasone and bortezomib."
5.	Bortezomib (Velcade)FDA Link 11/01/2011 - "Response to bortezomib of a patient with scleromyxedema refractory to other therapies." 10/01/2011 - "Scleromyxedema and dermato–neuro syndrome in a patient with multiple myeloma effectively treated with dexamethasone and bortezomib." 05/01/2012 - "The combination of bortezomib and dexamethasone is an efficient therapy for relapsed/refractory scleromyxedema: a rare disease with new clinical insights." 12/01/2020 - "Treatment of scleromyxedema with lenalidomide, bortezomib and dexamethasone: A case report and review of the literature." 12/01/2020 - "Triplet therapy with lenalidomide, bortezomib, and dexamethasone can be an effective therapy for scleromyxedema, especially in patients with refractory or relapsed disease."
6.	Adrenal Cortex Hormones (Corticosteroids)IBA 03/01/2006 - "We describe the case of a man with scleromyxedema treated with systemic corticosteroids whose skin lesions improved gradually within 4 weeks." 03/01/2006 - "Scleromyxedema: An experience using treatment with systemic corticosteroid and review of the published work." 01/01/2001 - "[Cutaneo-systemic papulosclerotic mucinosis (scleromyxedema): remission after extracorporeal photochemotherapy and corticoid bolus]." 10/16/2004 - "The presentation of encephalopathy as a stroke and its excellent response to treatment with corticoids pointed to a case of Hashimoto's encephalopathy, although we cannot completely rule out the possibility of it being a dermato-neuro syndrome, which is associated to scleromyxedema. "
7.	tranilastIBA 10/01/2021 - "Further studies are required to confirm the efficacy of tranilast for acral persistent papular mucinosis."
8.	Immunoglobulin G (IgG)IBA 05/22/1976 - "Only three other cases of papular mucinosis in which the IgG M component had K-type light chains have been reported. " 05/22/1976 - "A 59-year-old woman had papular mucinosis and IgG(K) M component in the serum. " 05/22/1976 - "Papular mucinosis with IgG(K) M component." 07/02/2020 - "Scleromyxedema (rare cutaneous mucinosis), is characterized by the formation of lichenoid papules and presence of Serum monoclonal IgG in most cases, or all; after repeated testing. " 03/01/2016 - "A male patient is presented with long-lasting paraproteinemia of monoclonal IgG λ, who suffered from recurrent, and until the last one, mostly reversible episodes of dermatoneuro syndrome, described exclusively in scleromyxedema. "
9.	Immunoglobulins (Immunoglobulin)IBA 03/01/2021 - "Monoclonal immunoglobulins (Ig) from the serum of 12 consecutive patients diagnosed with scleromyxedema (SME) were analyzed using electrophoretic and immunoblotting techniques. " 01/09/2020 - "Scleromyxedema is a progressive, systemic connective tissue disorder characterized by fibro-mucous skin lesions and increased serum monoclonal immunoglobulin levels. " 01/06/2015 - "[Scleromyxedema with neurological symptoms: successful treatment with immunoglobulins]." 04/01/1972 - "Idiotypic determinants on the monoclonal immunoglobulins associated with papular mucinosis." 01/01/1972 - "Immunochemical analysis of the basic immunoglobulin in papular mucinosis."
10.	Proteins (Proteins, Gene)FDA Link 05/01/1999 - "We describe a 40-year-old woman who had scleromyxedema associated with a monoclonal G lambda protein. " 03/01/1979 - "The fibrohistiocytic and mucinous change of the skin in scleromyxedema and often the monoclonal protein can be controlled by low-dose chemotherapy. " 03/01/1979 - "Scleromyxedema is an uncommon cutaneous fibromucinous disease with a monoclonal protein, which has resisted a number of therapies. " 01/01/2013 - "The differential diagnosis included scleredema and scleromyxedema; serum and urine protein electrophoresis were within normal limits. " 03/01/1983 - "Angioimmunoblastic lymphadenopathy with scleromyxedema-like lesions and serum monoclonal protein."

Therapies and Procedures

1.	Therapeutics 05/01/2012 - "We suggest this therapy as a suitable first-line treatment for scleromyxedema and for its neurological complications." 11/01/2018 - "Long-term maintenance treatment is usually required and close clinical follow-up is necessary as recurrence of scleromyxedema is common after withdrawal of an effective therapy." 12/31/2014 - "Ultimately, the patient did well with appropriate therapy, but his presentation illustrates the importance of more widespread familiarity among dermatologists and dermatopathologists with this variant of scleromyxedema. " 07/01/2013 - "Scleromyxedema: a multicenter study of characteristics, comorbidities, course, and therapy in 30 patients." 08/01/2007 - "Scleromyxedema: treatment of widespread cutaneous involvement by total skin electron-beam therapy."
2.	Stem Cell Transplantation 08/01/2001 - "Complete remission of scleromyxedema following autologous stem cell transplantation." 07/15/2006 - "Steady remission of scleromyxedema 3 years after autologous stem cell transplantation: an in vivo and in vitro study." 08/01/2022 - "Autologous stem cell transplantation in a patient with scleromyxedema." 02/01/2015 - "Allogeneic stem cell transplantation for the treatment of refractory scleromyxedema." 10/01/2016 - "High-dose melphalan with autologous stem cell transplantation appears to be a safe and effective long-term treatment in patients with scleromyxedema. "
3.	Drug Therapy (Chemotherapy) 10/01/2005 - "High-dose chemotherapy with stem cell rescue is feasible for patients with scleromyxedema and, although not curative, offers durable remission in most patients. " 10/01/2005 - "We retrospectively evaluated 6 patients who were offered high-dose chemotherapy with stem cell rescue as treatment for scleromyxedema. " 05/01/2000 - "The skin lesions of scleromyxedema cleared completely on treatment of seminoma with chemotherapy." 05/01/1993 - "Chemotherapy with various alkylating agents has been reported to be successful in the treatment of lichen myxedematosus. " 03/01/1979 - "The fibrohistiocytic and mucinous change of the skin in scleromyxedema and often the monoclonal protein can be controlled by low-dose chemotherapy. "
4.	Photopheresis 10/01/1991 - "Extracorporeal photochemotherapy: a potentially useful treatment for scleromyxedema." 01/01/2001 - "In the absence of controlled studies concerning the efficiency of the different drugs used in scleromyxedema, the main advantage of photopheresis lies in the safety of this procedure." 10/01/2001 - "Two patients with scleromyxedema were treated with extracorporeal photopheresis (ECP). " 10/01/2001 - "[Extracorporeal photopheresis--treatment option in scleromyxedema?]." 01/01/2001 - "[Cutaneo-systemic papulosclerotic mucinosis (scleromyxedema): remission after extracorporeal photochemotherapy and corticoid bolus]."
5.	Plasmapheresis 09/01/1997 - "[Papular mucinosis--successful therapy with plasmapheresis]." 05/01/1990 - "Successful treatment of scleromyxedema with plasmapheresis and immunosuppression." 06/01/1987 - "Plasmapheresis in a patient with scleromyxedema." 08/01/2012 - "Scleromyxedema with monoclonal gammopathy and neurological involvement: recovery from coma after plasmapheresis?" 08/01/2014 - "[Dermato-neuro syndrome during scleromyxedema: efficacy of plasmapheresis and intravenous immunoglobulin]."