Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema
It is also an endothelial marker and stains solitary fibrous tumor, spindle cell lipoma, superficial acral fibromyxoma, sclerotic fibroma, Kaposi sarcoma, neurofibroma, tricholemmoma, scleromyxedema
, and nephrogenic systemic fibrosis, among others.
is the generalized variant of lichen myxedematosus and is accompanied by papular lesions, diffuse sderosis and erythema.
Pope: Systemic sclerosis can be confused with such sclerodermalike skin disorders as eosinophilic fasciitis (EF), morphea, scleromyxedema
, and nephrogenic systemic fibrosis (NSF).
represents the skin thickening seen in patients with a gammopathy.
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.
Due to its similarities to scleromyxedema
and its occurrence only in patients undergoing dialysis, this new disorder was initially described as "scleromyxedema
-like cutaneous disease of dialysis" (Cowper, 2003, p.
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.
Although nephrogenic fibrosing dermopathy can somewhat resemble scleroderma or scleromyxedema
clinically, the face is typically spared in nephrogenic fibrosing dermopathy, unlike the other disorders, and paraproteinemia and autoantibodies are usually absent.
On clinical examination, the patients had fibrotic skin lesions histologically resembling scleromyxedema
on their distal extremities and trunk, resulting in severe contractions and limited mobility.