Transient efficacy of double high-dose chemotherapy and autologous peripheral stem cell transplantation, immunoglobulin, thalidomide, and bortezomib in the treatment of scleromyxedema.

Abstract

Scleromyxedema is a rare disorder characterized by mucin deposits in the dermis and monoclonal gammopathy. No definitive treatment of this condition has been described to date. We present the case of a 38-year-old male patient with scleromyxedema who underwent double consecutive autologous peripheral stem cell transplantations and received immunoglobulin, thalidomide, and bortezomib. This resulted in considerable clinical and pathologic amelioration of the patient's condition. However, 3 years after the second transplant, the patient relapsed and manifested the same skin lesions evident at his initial presentation.

• Authors: Selmin Ataergin, Fikret Arpaci, Murat Demiriz, Ahmet Ozet
• Journal: American journal of clinical dermatology (Am J Clin Dermatol) Vol. 9 Issue 4 Pg. 271-3 ( 2008) ISSN: 1175-0561 [Print] New Zealand
• PMID: 18572979 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antineoplastic Agents
• Boronic Acids
• Immunoglobulins
• Pyrazines
• Thalidomide
• Bortezomib

Topics

• Adult
• Antineoplastic Agents (therapeutic use)
• Boronic Acids (therapeutic use)
• Bortezomib
• Drug Therapy, Combination
• Humans
• Immunoglobulins (therapeutic use)
• Male
• Peripheral Blood Stem Cell Transplantation
• Pyrazines (therapeutic use)
• Recurrence
• Scleromyxedema (therapy)
• Thalidomide (therapeutic use)