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Pharmacotherapy of scleromyxedema
Expert Opinion on Orphan Drugs, 2013 Introduction: Scleromyxedema is a rare primary cutaneous mucinosis with a chronic and progressive course and possible severe systemic complications. There is no consensus on the optimal treatment for this potentially fatal disease because of the rarity of the disorder with limited number of case reports along with the lack of randomized controlled ...
CINOTTI, ELISA, Rongioletti, Franco
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Clinics in Dermatology, 2006
Scleromyxedema is a rare cutaneous mucinous disease characterized by a generalized papular sclerodermoid eruption and systemic manifestations that can lead to significant morbidity and mortality. Although its etiology remains unknown, most theories focus on a pathogenic role by paraproteins; it must be noted, however, that nonparaprotein factors have ...
Clara-Dina, Cokonis Georgakis +3 more
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Scleromyxedema is a rare cutaneous mucinous disease characterized by a generalized papular sclerodermoid eruption and systemic manifestations that can lead to significant morbidity and mortality. Although its etiology remains unknown, most theories focus on a pathogenic role by paraproteins; it must be noted, however, that nonparaprotein factors have ...
Clara-Dina, Cokonis Georgakis +3 more
+6 more sources
Journal of the American Academy of Dermatology, 1999
Scleromyxedema is a sclerotic variant of papular mucinosis, in which lichenoid papules and scleroderma-like features are present. We describe a patient with scleromyxedema with IgG type lambda chain paraprotein, a systemic sclerosis-like illness, and myositis.
Z, Bata-Csorgo +6 more
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Scleromyxedema is a sclerotic variant of papular mucinosis, in which lichenoid papules and scleroderma-like features are present. We describe a patient with scleromyxedema with IgG type lambda chain paraprotein, a systemic sclerosis-like illness, and myositis.
Z, Bata-Csorgo +6 more
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Archives of Dermatology, 1969
A 65-year-old woman developed typical scleromyxedema, with the associated M-type abnormal serum protein and marrow plasmacytosis. Treatment with systemic melphalan resulted in a dramatic response. The dermatosis improved noticeably after one month and a biopsy taken after five months of therapy showed almost total restitution to normal. The response of
T, Horio, I, Yonezawa
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A 65-year-old woman developed typical scleromyxedema, with the associated M-type abnormal serum protein and marrow plasmacytosis. Treatment with systemic melphalan resulted in a dramatic response. The dermatosis improved noticeably after one month and a biopsy taken after five months of therapy showed almost total restitution to normal. The response of
T, Horio, I, Yonezawa
+7 more sources
Journal of the American Academy of Dermatology, 1995
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 ...
A M, Dinneen, C H, Dicken
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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 ...
A M, Dinneen, C H, Dicken
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Histopathologic characteristics of scleromyxedema: A study of a series of 34 cases
Journal of the American Academy of Dermatology, 2016 Background Few histologic studies describe the histopathologic aspects of scleromyxedema. Objective We sought to describe the histopathologic and immunohistochemical features of scleromyxedema in a large series of patients.
Franco Rongioletti +2 more
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Journal of Neurology, 2019
Scleromyxedema is a chronic, idiopathic disorder associated with monoclonal gammopathy, and characterized by dermal mucin deposition. However, systemic manifestations are frequent, including neuromuscular symptoms. We herein present a 71-year-old man who developed a vacuolar myopathy in a context of a known scleromyxedema, and we compare our ...
Antoine Soulages +9 more
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Scleromyxedema is a chronic, idiopathic disorder associated with monoclonal gammopathy, and characterized by dermal mucin deposition. However, systemic manifestations are frequent, including neuromuscular symptoms. We herein present a 71-year-old man who developed a vacuolar myopathy in a context of a known scleromyxedema, and we compare our ...
Antoine Soulages +9 more
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Encephalopathy in scleromyxedema
Neurology, 1999The authors monitored CSF findings for over 5 months in a patient with a fatal case of scleromyxedema and two episodes of encephalopathy. During both encephalopathy episodes, CSF protein and immunoglobulin G (IgG) levels were elevated without an increased IgG index or IgG synthesis rate. A CSF-dominant increase in the concentration of interleukin-6 (IL-
K, Johkura +4 more
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