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Scleromyxedema: a scleroderma-like disorder with systemic manifestations
S E Gabriel1, H O Perry, G B Oleson
1Department of Rheumatology, Mayo Clinic, Rochester, Minnesota 55905.
Medicine
|January 1, 1988
Summary
Scleromyxedema, a rare connective tissue disease, often involves systemic issues beyond skin lesions. Alkylating agents show limited efficacy for extracutaneous symptoms and carry significant risks, including sepsis and malignancy.
Area of Science:
- Rheumatology
- Dermatology
- Pathology
Background:
- Scleromyxedema is a rare fibromucinous connective tissue disorder.
- It is characterized by papular skin lesions, sclerosis, and serum monoclonal gammopathy.
- Limited data exists on its natural history and systemic manifestations.
Observation:
- A review of 19 patients (1950-1985) with biopsy-proven scleromyxedema identified systemic involvement.
- Common symptoms included dysphagia, proximal muscle weakness, and dyspnea on exertion.
- Pulmonary dysfunction (reduced diffusing capacity, volumes) and cor pulmonale were observed.
Findings:
- Monoclonal gammopathy was present in 13 patients.
- Three patients had inflammatory myopathy, and one showed "scleroderma kidney" changes.
- Melphalan treatment led to skin regression in some but not extracutaneous manifestations, with significant toxicity (sepsis, malignancy).
Implications:
- Systemic manifestations in scleromyxedema are more common than previously thought and can mimic scleroderma.
- Alkylating agents pose substantial risks and should be reserved for severe cases.
- Further research is needed to establish optimal therapeutic strategies for scleromyxedema.