Papular mucinosis, destructive arthropathy, median neuropathy, and sicca complex.
A patient with papular mucinosis (scleromyxedema) developed an erosive seronegative rheumatoid-like arthropathy, carpal tunnel syndrome, sicca complex, and marked increase in TG(OKT8) suppressor/cytotoxic circulating T-cells akin to that reported in scleroderma. Sclerodactyly, acrolysis and stiff digits were striking but other features of scleroderma, i.e., Raynaud's and esophageal hypoperistalsis, were absent. The diagnosis of papular mucinosis, a pseudoscleroderma syndrome, should be considered in a patient with atypical arthritis, median neuropathy, myopathy, and/or sclerodactyly and a papular lichenoid dermatopathy. Skin mucin stain and the demonstration of the distinct serum paraprotein (PM-spike) are confirmatory. We stress the salient diagnostic clinical features of the leonine-like facies of papular mucinosis.
D001327:Autoimmune Diseases / Q000150:complications; D005260:Female; D006801:Humans; D007592:Joint Diseases / Q000150:complications*; D007958:Leukocyte Count; D008875:Middle Aged; D009077:Mucins / Q000378:metabolism; D009230:Myxedema / Q000150:complications*; D010292:Paresthesia / Q000150:complications*; D012598:Sclerosis; D012859:Sjogren's Syndrome / Q000150:complications*; D013602:T-Lymphocytes, Cytotoxic; D050378:T-Lymphocytes, Regulatory
D009077:Mucins