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Scleromyxedema: a complete response to prednisone
1Division of Hematology and Internal Medicine, Mayo Clinic Rochester, Minnesota 55905, USA.
Mayo Clinic Proceedings
|May 13, 1999
Summary
Scleromyxedema, a rare skin disorder, involves mucin buildup and monoclonal proteins. Oral prednisone effectively treated a patient
Area of Science:
- Dermatology
- Immunology
- Internal Medicine
Background:
- Scleromyxedema is a rare mucinosis characterized by skin deposition of mucin.
- It is invariably associated with a serum monoclonal protein.
- Systemic manifestations can significantly impact patient morbidity.
Observation:
- A 40-year-old woman presented with scleromyxedema and a monoclonal G lambda protein.
- She experienced severe systemic symptoms including fatigue, esophageal dysmotility, and myopathy.
- These symptoms resolved completely with oral prednisone treatment.
Findings:
- The patient achieved a 24-month clinical remission after discontinuing prednisone.
- This case suggests oral corticosteroids may be an effective initial therapy for scleromyxedema.
- Complete symptom resolution was observed with prednisone.
Implications:
- Oral corticosteroid therapy presents a potentially safer alternative to alkylating agents for scleromyxedema.
- This approach may reduce treatment-associated morbidity and mortality.
- Alkylating agents could be reserved for refractory cases, improving patient outcomes.