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: 188 relevant articles (24 outcomes, 2 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Mucinoses
2.	Multiple Myeloma
3.	Hypothyroidism
4.	Seminoma
5.	Acute Myelomonocytic Leukemia

Experts

1.	Hummers, Laura K: 3 articles (11/2014 - 01/2008)
2.	Rongioletti, F: 3 articles (03/2001 - 10/2000)
3.	Rebora, A: 3 articles (03/2001 - 10/2000)
4.	Stetsenko, Galina Y: 2 articles (03/2015 - 08/2008)
5.	Vary, Jay C: 2 articles (03/2015 - 08/2008)
6.	Guillet, G: 2 articles (08/2014 - 04/2009)
7.	Kreuter, Alexander: 2 articles (10/2005 - 03/2005)
8.	Schroeter, Arnold L: 2 articles (10/2005 - 01/2005)
9.	Ross, Edward V: 1 article (08/2015)
10.	Lloyd, Amanda A: 1 article (08/2015)

Drugs and Biologics

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

1.	Immunoglobulins (Immunoglobulin)IBA 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. " 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." 04/01/2009 - "[Efficacy of intravenous immunoglobulin in Arndt-Gottron scleromyxedema]." 01/06/2015 - "[Scleromyxedema with neurological symptoms: successful treatment with immunoglobulins]."
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." 05/01/2008 - "Scleromyxedema--thalidomide therapy."
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." 07/01/2002 - "A 74-year old patient with initial scleromyxedema is described, in whom a pulse therapy with melphalan was initiated already in the early stages of the disease. " 11/01/1984 - "[Lichen myxedematosus, I. Statistical studies on associated liver injury and successful treatment with melphalan]." 11/01/1979 - "Morbidity and mortality due to melphalan used in the treatment of scleromyxedema have been reported, therefore, this therapy should be reserved for only the most extreme cases, if at all." 03/01/1979 - "Treatment of scleromyxedema with melphalan."
4.	Intravenous Immunoglobulins (IVIG)FDA Link 06/01/2007 - "We report on a 56-year-old women with scleromyxedema, whose skin findings markedly improved following high-dose intravenous immunoglobulin therapy (Sandoglobulin((R)), 2 g/KG). " 05/01/2012 - "[Good response of scleromyxedema and dermato-neuro syndrome to treatment with intravenous immunoglobulins]." 07/01/2005 - "IVIG appears to be the therapy of choice for scleromyxedema. " 12/01/2008 - "IVIG and thalidomide in combination may be an effective novel treatment of scleromyxedema. " 08/01/2013 - "Combination of intravenous immunoglobulins and lenalidomide in the treatment of scleromyxedema."
5.	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." 08/01/2004 - "We report a case of a patient with scleromyxedema limited to the skin with an associated IgG lambda monoclonal protein treated successfully with high-dose 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."
6.	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." 01/01/2008 - "We present the case of a 38-year-old male patient with scleromyxedema who underwent double consecutive autologous peripheral stem cell transplantations and received immunoglobulin, thalidomide, and bortezomib. " 01/01/2008 - "Transient efficacy of double high-dose chemotherapy and autologous peripheral stem cell transplantation, immunoglobulin, thalidomide, and bortezomib in the treatment of scleromyxedema."
7.	Immunoglobulin G (IgG)IBA 01/01/2007 - "We report 2 cases of scleromyxedema, both associated with an IgG-lambda paraprotein. " 01/18/2002 - "However, the usual IgG lambda paraprotein associated with scleromyxedema was not observed in these patients. " 07/01/1982 - "[Clinico-evolutive development of a case of papular mucinosis associated with IgG-lambda paraprotein]." 03/01/1974 - "Papular mucinosis (scleromyxoedema of Arndt-Gottron) associated with paraprotein IgG kappa type." 01/01/1971 - "Isolation and characterization of the basic, diagnostic IgG globulin in the rare gammopathy, papular mucinosis."
8.	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." 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. "
9.	CladribineFDA LinkGeneric 08/01/1996 - "Treatment of scleromyxedema with 2-chlorodeoxyadenosine." 08/01/1996 - "We describe a patient with scleromyxedema who was treated successfully with 2-chlorodeoxyadenosine (2-CdA), a purine analog used mainly in the treatment of lymphoproliferative disorders."
10.	Triamcinolone Acetonide (Azmacort)FDA LinkGeneric 01/01/2010 - "From laboratory and histopathological findings, nodular-type lichen myxedematosus was diagnosed and nodules showed complete remission following local injection of triamcinolone acetonide."

Therapies and Procedures

1.	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. " 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. " 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. "
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." 02/01/2015 - "Allogeneic stem cell transplantation for the treatment of refractory scleromyxedema." 01/15/2006 - "Scleromyxedema: role of high-dose melphalan with autologous stem cell transplantation." 01/01/2008 - "We present the case of a 38-year-old male patient with scleromyxedema who underwent double consecutive autologous peripheral stem cell transplantations and received immunoglobulin, thalidomide, and bortezomib. "
3.	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]."
4.	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]."
5.	Transplants (Transplant) 08/01/2008 - "Spontaneous resolution of facial papular mucinosis in a transplant patient." 12/01/2014 - "Dermato-neuro syndrome in a patient treated with autologous stem cell transplant for scleromyxedema." 10/01/2005 - "Patients with renal failure have been identified recently, some on dialysis, others with renal transplants, who have scleromyxedema-like skin changes. "