Scleromyxedema is the generalized variant of lichen myxedematosus and is accompanied by papular lesions, diffuse sderosis and erythema.
Key Words: IVIG, monoclonal gammopathy, scleromyxedema, treatment
Pope: Systemic sclerosis can be confused with such sclerodermalike skin disorders as eosinophilic fasciitis (EF), morphea, scleromyxedema
, and nephrogenic systemic fibrosis (NSF).
has a clinical distribution that favors the head and neck region, and is almost always associated with a circulating paraprotein.
Systemic sclerosis must be differentiated from other conditions associated with skin fibrosis, including localized scleroderma, scleredema, scleromyxedema
, and eosinophilic fasciitis, as well as rare conditions (e.
Adult stem cells have shown themselves to be effective for spinal cord injury, Parkinson disease, ameliorating retinal degeneration (thus giving hope for treatment for diabetic retinopathy and age-related macular degeneration), as an adjunct for cancer chemotherapy (to replace the patient's hematopoietic system or for anemia), for various autoimmune conditions (including scleromyxedema
, multiple sclerosis and Crohn disease), metabolic disorders (such as Krabbe disease), bone repair protocols and repair of cardiac damage.
of cases Multiple myeloma 330 NSMM 20 Osteosclerotic myeloma 15 SMM 72 Indolent/evolving myeloma 8 Plasmacytoma (solitary) 22 Extramedullary myeloma 5 Multiple solitary plasmacytoma 3 Macroglobulinemia 9 IgM lymphoproliferative disease 2 IgM lymphoma 5 Smoldering macroglobulinemia 2 Primary systemic amyloidosis 269 LCDD 7 MGUS 114 Idiopathic Bence Jones proteinuria 4 Heavy chain disease 2 Cryoglobulinemia 4 Acquired Fanconi syndrome 3 Scleromyxedema
2 Plasma cell leukemia 1 Table 2.
The disorder, scleromyxedema
, is similar to scleroderma, a chronic connective tissue disease in which the skin thickens and becomes shiny and stiff.
In our patient, the clinical appearance of multiple tiny papules in linear patterns on an edematous background is characteristic of the papular form of scleromyxedema
Jolles S, Hughes J: Use of IVIG in the treatment of atopic dermatitis, urticaria, scleromyxedema
, pyoderma gangrenosum, psoriasis and pretibial myxedema.
The most important histologic differential diagnosis is with scleromyxedema
can produce a similar fibroblastic picture, but again, there is a key differentiation since patients with nephrogenic fibrosing dermopathy have no associated IgG-[lambda] paraprotein.