Skin Papules, Hand Neuropathy and Monoclonal Gammopathy

By Soumya Chatterjee, MD, and Anthony P. Fernandez, MD, PhD

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A 53-year-old man presented to the rheumatology clinic with a three-year history of an itchy rash, Raynaud’s phenomenon, dysphagia and a burning sensation in his hands.

Physical examination was notable for firm, greasy papules across his forehead that led to the formation of glabellar grooves (A).

Greasy papules on the patient's forehead led to the formation of glabellar grooves — A. Papules across the forehead led to the formation of glabellar grooves.

There were waxy papules on his hands with associated skin thickening and finger flexion contractures (B., above). Similar skin changes were seen on his nose, lips, ears, trunk and feet. There was no telangiectasia or calcinosis.

Sensory neuropathy was present in his hands, arms and face. Tests of thyroid function were normal. Serum protein electrophoresis with immunofixation identified an IgG-λ monoclonal gammopathy, and a bone marrow biopsy was normal.

A subsequent skin-biopsy sample obtained from the right side of the neck showed dermal spindle-cell proliferation, thickened collagen fibers, fibrosis and perivascular inflammation (C, hematoxylin and eosin stain), as well as increased dermal mucin deposition (D, colloidal iron stain). A diagnosis of scleromyxedema was made.

Skin biopsy from the neck — C: A skin biopsy sample from the neck showed dermal spindle-cell proliferation, thickened collagen fibers, fibrosis and perivascular inflammation.

increased dermal mucin deposition — D. Increased dermal mucin deposition.

Scleromyxedema is a primary cutaneous mucinosis typically associated with a paraproteinemia. This type of sclerosing skin disorder may cause extracutaneous symptoms, as was seen in this patient.

Although infusions of intravenous immune globulin provided minimal relief initially, treatment with lenalidomide resulted in abatement of symptoms and reduction in paraproteinemia after four months.

This article was originally published in The New England Journal of Medicine, Nov. 23, 2023.