SCLEROMYXEDEMA (PARAPROTEINEMIA ASSOCIATED DESMOPLASIA)
This 75 year old male developed thickening and induration of both upper extremities beginning about 5 months ago. He went into renal failure about 7 months, ago, but the nature of his renal disease is not known at the present time. Following this biopsy, serum protein electrophoresis was done, and an abnormal spike was noted. He has no evidence of multiple myeloma. The clinical findings are characteristic of scleromyxedema.

Every time I see a biopsy of this condition or of the related lichen myxedematosus, my first impression is that I am dealing with some type of hyperplasia of spindle cells or with a spindle cell neoplasm such as a desmoplastic melanoma that is composed of dispersed, minimally atypical spindle cells. The myxoid component, which is stressed in the literature, may just be a reflection of the immaturity of collagenous tissue, and it may not be apparent. Actual fibrosis of the dermis and subcutis can be seen. Someone should give this condition a new name such as 'paraproteinemia associated desmoplasia' (PAD) or 'paraproteinemia associated fibroplasia' (PAF) to better reflect what is really going on.


Initial biopsy:
Scan showing dermal involvement plus septal fibrosis of the subcutis.
Low power of dermis. There was no evidence of the radial growth phase of a melanoma in any of the sections.
High power of above. The basophilia is due to solar elastosis. An alcian blue stain at a ph of 2.5 failed to demonstrate increased tissue mucin.
Low power of thickened fibrous septum in subcutis.
High power of above.

A second biopsy was done from a different location on the same arm:
 
Scan showing dermal involvement and massive fibrosis of the subcutis.
Low power of dermis showing solar elastosis and increased number of fibroblasts. A colloidal iron stain for tissue mucin showed a few small foci where this was increased.
Low power of subcutis. The collagen fibers are smaller and more randomly oriented than those that would be expected in scleroderma or in eosinophilic fasciitis. No eosinophiles were noted.
High power of above showing increased fibroblasts and eosinophilic collagen.

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