Scleromyxedema may occasionally resemble scleroderma.

Features shared by scleromyxedema and scleroderma:

(1) Raynaud's phenomenon

(2) sclerodactyly

(3) esophageal dysmotility

(4) myopathy

(5) telangiectasia

(6) skin thickening


Features useful for recognizing scleromyxedema:

(1) numerous skin papules

(2) monoclonal gammopathy

(3) dermal mucinous material in skin biopsy

(4) skin movable over subcutaneous tissues with deep skin folds ("Shar-Pei sign")

(5) leonine facies and deposits in the external ear


Situations where the diagnosis may be more difficult:

(1) papular eruption inapparent

(2) failure to biopsy

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