melphalan and Myxedema

Scleromyxedema is a rare fibromucinous disorder that is often difficult to treat and that is associated with significant morbidity and mortality.. Our purpose was to study the natural history of the disease and its response to therapy with alkylating agents.. A clinicopathologic review of 26 patients with scleromyxedema was performed, and the extracutaneous findings and response to therapy with alkylating agents were noted.. Extracutaneous manifestations, most often gastrointestinal, were present in 20 of 26 patients. An abnormal paraprotein was found in 23 of 26 patients, most commonly IgG-lambda (18 patients). Melphalan was used as therapy for 17 patients. The disease proved fatal in 10 of the treated patients.. The overall prognosis in scleromyxedema is poor. Therapy is difficult. Although alkylating agents may prove beneficial in the short term, significant toxicity of these agents is apparent with long-term use.

Topics: Adult; Aged; Female; Follow-Up Studies; Humans; Male; Melphalan; Middle Aged; Myxedema; Sclerosis; Skin

Scleromyxedema, the most severe manifestation of the spectrum of lichen myxedematosus, is characterized by cutaneous mucinosis, extracutaneous manifestations, and a monoclonal gammopathy. Seven of 8 patients evaluated at our center were treated with high-dose melphalan (180 mg/m(2) intravenously) and autologous peripheral blood stem cell transplantation, with marked improvement of gastrointestinal, central nervous system, pulmonary manifestations, and Karnofsky performance status. Five patients obtained a cutaneous complete remission and 2 patients had partial remissions. Three patients with slight progression in the skin at 12, 8, and 4 months after treatment received a second cycle of high-dose melphalan and had further symptomatic improvement. The lichen myxedematosus-scleromyxedema spectrum appears to be a continuum that requires the presence of a serum paraprotein and differs in severity of skin lesions, extracutaneous manifestations, and performance status. High-dose melphalan followed by autologous transplantation appears effective for improving the symptoms and systemic manifestations of scleromyxedema.

Topics: Adult; Aged; Antineoplastic Agents, Alkylating; Dose-Response Relationship, Drug; Female; Hematopoietic Stem Cell Transplantation; Humans; Male; Maximum Tolerated Dose; Melphalan; Middle Aged; Myxedema; Palliative Care; Remission Induction; Scleroderma, Limited; Scleroderma, Systemic; Transplantation, Autologous; Treatment Outcome

Scleromyxedema is a rare chronic fibromucinous disorder that can have devastating clinical manifestations, including sclerosis of the skin with progressive pharyngeal and upper airway involvement, resulting in high mortality due to respiratory complications. Herein we describe a novel therapeutic approach. Because autologous hematopoietic stem cell transplantation is effective in other plasma cell proliferative disorders, it may be effective in this setting.. We retrospectively evaluated 6 patients who were offered high-dose chemotherapy with stem cell rescue as treatment for scleromyxedema. One heavily pretreated patient was unable to mobilize stem cells. The remaining 5 patients mobilized stem cells and underwent successful transplantation. There was no treatment-related mortality. Hematologic responses were seen in 4 patients, including 2 complete remissions and 2 partial remissions, and all 4 had improvement in extracutaneous manifestations. All 4 patients subsequently had relapse of the monoclonal protein, and 3 developed skin relapses at 14, 37, and 45 months.. High-dose chemotherapy with stem cell rescue is feasible for patients with scleromyxedema and, although not curative, offers durable remission in most patients. This therapy should be considered before treatment with alkylating agents or other treatments that could adversely affect the ability to collect stem cells.

Topics: Adult; Dose-Response Relationship, Drug; Female; Hematopoietic Stem Cell Mobilization; Humans; Lichenoid Eruptions; Male; Melphalan; Middle Aged; Myxedema; Peripheral Blood Stem Cell Transplantation; Remission Induction; Retrospective Studies; Salvage Therapy; Scleroderma, Limited; Transplantation, Autologous; Treatment Outcome

Scleromyxedema is a rare mucinous disease without thyroid dysfunction, associated with a monoclonal gammopathy. The deposits of mucin in the skin and other organs such as the cardiovasculary system, determine the prognosis of the disease. 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. Four cycles of melphalan in combination with prednisolon led to a complete and sofar stable remission of the disease.

Topics: Aged; Biopsy; Diagnosis, Differential; Drug Therapy, Combination; Facial Dermatoses; Female; Humans; Lichenoid Eruptions; Melphalan; Myxedema; Prednisolone; Skin

Topics: Aged; Etretinate; Humans; Male; Melphalan; Myxedema; Radiography; Recurrence

Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Cyclophosphamide; Humans; Leg Dermatoses; Male; Mechlorethamine; Melphalan; Methotrexate; Myxedema

We report two cases of scleromyxedema treated with melphalan (alkeran) and dermabrasion, both patients with central nervous system involvement. In the first case, herpetic encephalitis, possibly due to the immunoglobulin disturbance, preceded the skin changes. In the second case, impaired cerebral function was caused by a meningeoma. In the first case of short duration it seems as if progression of the disease has been arrested. In the second, of more than 26 years duration the skin changes have seemed unchanged, sclerotic for some years, though some improvement was noticed following dermabrasion.

Topics: Adult; Aged; Dermabrasion; Female; Humans; Melphalan; Myxedema; Skin Diseases

Topics: Female; Humans; Leukemia, Erythroblastic, Acute; Melphalan; Middle Aged; Myxedema; Skin Diseases

An unusual, nodulocystic form of scleromyxedema (lichen myxedematosus) developed in a 48-year-old man with a six-year history of psoriasis. The scleromyxedema responded to intermittent therapy with melphalan and prednisone. Dermabrasion smoothed and softened the skin and increased the mobility of the perioral skin. Two months after remission of the skin lesions, psoriasis recurred.

Topics: Dermabrasion; Humans; Male; Melphalan; Middle Aged; Mucopolysaccharidoses; Myxedema; Paraproteinemias; Prednisone; Psoriasis; Recurrence; Skin Diseases

Topics: Adult; Blood Protein Disorders; Blood Protein Electrophoresis; Bone Marrow; Bone Marrow Cells; Brain Edema; Bronchopneumonia; Chondroitin; Culture Techniques; Fluorescent Antibody Technique; Glycoproteins; Humans; Hyaluronic Acid; Immunoelectrophoresis; Immunoglobulin G; Male; Melphalan; Myxedema; Skin; Skin Diseases; Sulfuric Acids

Topics: Adult; Aged; Blood Protein Disorders; Diagnosis, Differential; Female; Humans; Immunoglobulin G; Immunoglobulins; Immunosuppressive Agents; Male; Melphalan; Methotrexate; Middle Aged; Multiple Myeloma; Myxedema; Scleroderma, Systemic; Serum Globulins; Skin Diseases

Topics: Aged; Biopsy; Female; gamma-Globulins; Humans; Immunoelectrophoresis; Melphalan; Myxedema; Skin; Skin Diseases